<HashMap><database>MetaboLights</database><file_versions><headers><Content-Type>application/xml</Content-Type></headers><body><files><Tabular>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/m_MTBLS14628_LC-MS_negative_reverse-phase_v2_maf.tsv</Tabular><Tabular>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/m_MTBLS14628_LC-MS_positive_reverse-phase_v2_maf.tsv</Tabular><Txt>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/s_MTBLS14628.txt</Txt><Txt>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/i_Investigation.txt</Txt><Txt>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/a_MTBLS14628_LC-MS_negative_reverse-phase.txt</Txt><Txt>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628/a_MTBLS14628_LC-MS_positive_reverse-phase.txt</Txt></files><type>primary</type></body><statusCode>OK</statusCode><statusCodeValue>200</statusCodeValue></file_versions><scores/><additional><ftp_download_link>ftp://ftp.ebi.ac.uk/pub/databases/metabolights/studies/public/MTBLS14628</ftp_download_link><metabolite_identification_protocol>&lt;p>Metabolite identification was performed using MSDIAL software. The extracted features were annotated by accurate mass matching with a mass tolerance of &amp;lt;10 ppm and MS/MS spectral matching with a mass tolerance of &amp;lt;0.01 Da. The annotation was conducted against public metabolite databases, including HMDB, MassBank, and GNPS, as well as the in-house Bioprofile metabolite standards database (BP-DB).&lt;/p></metabolite_identification_protocol><repository>MetaboLights</repository><study_status>Public</study_status><ptm_modification></ptm_modification><instrument_platform>Liquid Chromatography MS - negative - reverse-phase</instrument_platform><instrument_platform>Liquid Chromatography MS - positive - reverse-phase</instrument_platform><chromatography_protocol>&lt;p>Chromatographic separation was performed using a SHIMADZU-LC30 ultra-high-performance liquid chromatography system. Samples were analyzed on an ACQUITY UPLC HSS T3 column (2.1 × 100 mm, 1.8 μm; Waters, Milford, MA, USA). During analysis, the samples were maintained in the autosampler at 4°C. The injection volume was 4 μL, the column temperature was 40°C, and the flow rate was 0.3 mL/min. Mobile phase A was 0.1% formic acid in water, and mobile phase B was acetonitrile. The gradient elution program was as follows: 0–2 min, 0% B; 2–6 min, 0–48% B; 6–10 min, 48–100% B; 10–12 min, 100% B; 12–12.1 min, 100–0% B; and 12.1–15 min, 0% B.&lt;/p></chromatography_protocol><publication>Genetic Ablation of Sfxn5 Induces Mitochondrial Dysfunction and Precipitates Lethal Metabolic Crisis in Mice.</publication><submitter_affiliation>Peking University</submitter_affiliation><submitter_name>Duo Keai</submitter_name><organism_part>Liver</organism_part><organism_part>brain</organism_part><technology_type>mass spectrometry assay</technology_type><disease></disease><extraction_protocol>&lt;p>Tissue samples were thawed at 4°C, and 50 mg of each sample was transferred into an EP tube. Three medium steel beads and 200 μL of pre-cooled 80% methanol aqueous solution were added, and the samples were homogenized using a tissue disruptor. Subsequently, 800 μL of pre-cooled 80% methanol aqueous solution was added, followed by ultrasonic extraction in an ice bath for 20 min. The samples were then incubated at 20°C for 1 h and centrifuged at 16,000 × g for 20 min at 4°C. The supernatant was collected and dried using a high-speed vacuum concentrator. Before mass spectrometry analysis, the dried extracts were reconstituted in 100 μL methanol/water solution (1:1, v/v), centrifuged at 20,000 × g for 15 min at 4°C, and the supernatant was used for analysis.&lt;/p></extraction_protocol><organism>Mus musculus</organism><full_dataset_link>https://www.ebi.ac.uk/metabolights/MTBLS14628</full_dataset_link><author>Chunguang Ren. Chongqing Medical University. chunguangren@cqmu.edu.cn.</author><author>Huan Zhang. Chongqing Medical University. 191109@hospital.cqmu.edu.cn.</author><data_transformation_protocol>&lt;p>Raw data were processed using MSDIAL software for peak alignment, retention time correction, and peak area extraction. Metabolite annotation was performed by matching accurate mass with a mass tolerance of &amp;lt;10 ppm and MS/MS spectra with a mass tolerance of &amp;lt;0.01 Da against public databases, including HMDB, MassBank, and GNPS, as well as the in-house Bioprofile metabolite standards database (BP-DB). Ion peaks with more than 50% missing values within a group were removed before statistical analysis. Positive- and negative-ion data were normalized separately by total peak area, integrated, and then analyzed using Python software. The data were preprocessed by unit variance scaling before subsequent data analysis.&lt;/p></data_transformation_protocol><study_factor>Group</study_factor><submitter_email>keaiduoduo998@126.com</submitter_email><sample_collection_protocol>&lt;p>Brain and liver tissues were harvested from WT and Sfxn5-knockout mouse pups at postnatal day 26. Samples were immediately snap-frozen in liquid nitrogen after collection and stored at 80°C until analysis. Six biological replicates were analyzed for each tissue and genotype group, including WT brain, Sfxn5-knockout brain, WT liver, and Sfxn5-knockout liver. Prior to metabolite extraction, tissue samples were thawed at 4°C, and approximately 50 mg of each sample was processed.&lt;/p></sample_collection_protocol><omics_type>Metabolomics</omics_type><study_design>Metabolomics</study_design><study_design>SHIMADZU-LC30 UHPLC</study_design><study_design>Mus musculus</study_design><study_design>untargeted analysis</study_design><study_design>Liver</study_design><study_design>Urea cycle</study_design><study_design>tissue</study_design><study_design>Metabolism</study_design><study_design>brain</study_design><study_design>Sfxn5</study_design><study_design>Hepatic metabolic crisis</study_design><study_design>Mitochondrial disorder</study_design><study_design>Thermo Scientific QE Plus</study_design><curator_keywords>Metabolomics</curator_keywords><curator_keywords>SHIMADZU-LC30 UHPLC</curator_keywords><curator_keywords>Mus musculus</curator_keywords><curator_keywords>untargeted analysis</curator_keywords><curator_keywords>Liver</curator_keywords><curator_keywords>Urea cycle</curator_keywords><curator_keywords>tissue</curator_keywords><curator_keywords>Metabolism</curator_keywords><curator_keywords>brain</curator_keywords><curator_keywords>Sfxn5</curator_keywords><curator_keywords>Hepatic metabolic crisis</curator_keywords><curator_keywords>Mitochondrial disorder</curator_keywords><curator_keywords>Thermo Scientific QE Plus</curator_keywords><mass_spectrometry_protocol>&lt;p>Mass spectrometric analysis was performed using a QE Plus mass spectrometer (Thermo Scientific) equipped with a HESI source. Each sample was analyzed in both positive and negative electrospray ionization modes after UPLC separation. The ionization parameters were as follows: spray voltage, 3.8 kV in positive mode and 3.2 kV in negative mode; capillary temperature, 320°C; sheath gas, 30; auxiliary gas, 5; probe heater temperature, 350°C; and S-lens RF level, 50. The mass spectrometry acquisition time was 15 min, and the precursor ion scan range was 70–1050 m/z. The MS1 resolution was 70,000 at m/z 200, with an AGC target of 3e6 and a maximum injection time of 100 ms. For MS/MS acquisition, the 10 most intense precursor ions were selected for MS2 scans after each full scan. The MS2 resolution was 17,500 at m/z 200, with an AGC target of 1e5, a maximum injection time of 50 ms, HCD activation, an isolation window of 2 m/z, and stepped normalized collision energies of 20, 30, and 40.&lt;/p></mass_spectrometry_protocol><metabolite_name>2-((3-ethoxy-3-oxopropanoyl)oxy)-N,N,N-trimethylethan-1-aminium</metabolite_name><metabolite_name>6-Methoxy-pyridine-3-carboxylic acid</metabolite_name><metabolite_name>Spectral Match to Phe-Tyr from NIST14</metabolite_name><metabolite_name>Plantagonine</metabolite_name><metabolite_name>p-Mentha-1,3,5,8-tetraene</metabolite_name><metabolite_name>Corticosterone</metabolite_name><metabolite_name>Oct-5-enedioylcarnitine</metabolite_name><metabolite_name>Oroxin B</metabolite_name><metabolite_name>N-Fructosyl isoleucine</metabolite_name><metabolite_name>Guaiacol</metabolite_name><metabolite_name>Ectoine</metabolite_name><metabolite_name>2,5-Dimethyl-3-hexyne-2,5-diol</metabolite_name><metabolite_name>Albuterol</metabolite_name><metabolite_name>(2S,4R)-4-hydroxy-1-[(2S)-pyrrolidine-2-carbonyl]pyrrolidine-2-carboxylic acid</metabolite_name><metabolite_name>Citrulline</metabolite_name><metabolite_name>1,5-Naphthalenediamine</metabolite_name><metabolite_name>2,3-Dimethyl-2-cyclohexen-1-one</metabolite_name><metabolite_name>2-(2-aminoacetamido)-3-methylpentanoic acid</metabolite_name><metabolite_name>5-Hydroxy-DL-lysine</metabolite_name><metabolite_name>Spectral Match to Phe-Pro from NIST14</metabolite_name><metabolite_name>Ademetionine</metabolite_name><metabolite_name>N-Benzoyl-L-glutamic acid</metabolite_name><metabolite_name>Cyclopentylamine</metabolite_name><metabolite_name>Prostaglandin E2</metabolite_name><metabolite_name>7b,9-Dihydroxy-3-(hydroxymethyl)-1,1,6,8-tetramethyl-5-oxo-1,1a,1b,4,4a,5,7a,7b,8,9-decahydro-9ah-cyclopropa[3,4]benzo[1,2-e]azulen-9a-yl acetate</metabolite_name><metabolite_name>Cyclododecalactam</metabolite_name><metabolite_name>Spectral Match to Lys-Ile from NIST14</metabolite_name><metabolite_name>2,4-Thiazolidinedicarboxylic acid, 2-methyl-</metabolite_name><metabolite_name>Arginine</metabolite_name><metabolite_name>Fusaric acid</metabolite_name><metabolite_name>Phenylalanine methyl ester</metabolite_name><metabolite_name>Dimethylaminopropyl Lauramide</metabolite_name><metabolite_name>2,8-Dihydroxyquinoline</metabolite_name><metabolite_name>Phenylalanylserine</metabolite_name><metabolite_name>FMN</metabolite_name><metabolite_name>Unknown (carbon number 7)</metabolite_name><metabolite_name>12,13-Dhome</metabolite_name><metabolite_name>gamma-Glutamylphenylalanine</metabolite_name><metabolite_name>Lidocaine</metabolite_name><metabolite_name>Kainic acid</metabolite_name><metabolite_name>2-Cyclopenten-1-one</metabolite_name><metabolite_name>Pantothenic acid</metabolite_name><metabolite_name>1-Stearoyl-2-arachidonoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>Xanthurenic acid</metabolite_name><metabolite_name>Prostaglandin F28a</metabolite_name><metabolite_name>Bicifadine</metabolite_name><metabolite_name>beta-Ureidoisobutyric acid</metabolite_name><metabolite_name>N-Acetylmethionine, DL-</metabolite_name><metabolite_name>Acetoacetic acid</metabolite_name><metabolite_name>Leucyl-Leucine</metabolite_name><metabolite_name>Glycylvaline</metabolite_name><metabolite_name>Indole-3-acetyl-alanine</metabolite_name><metabolite_name>Polyornithine</metabolite_name><metabolite_name>4-Methylphthalic anhydride</metabolite_name><metabolite_name>Spectral Match to Leu-Thr from NIST14</metabolite_name><metabolite_name>Pyridine</metabolite_name><metabolite_name>4-Hydroxyquinoline</metabolite_name><metabolite_name>Spectral Match to Eicosapentaenoyl PAF C-16 from NIST14</metabolite_name><metabolite_name>1-Stearoyl-2-linoleoylphosphatidylcholine</metabolite_name><metabolite_name>O-Propanoyl-D-carnitine</metabolite_name><metabolite_name>N-acetyl-d-tryptophan</metabolite_name><metabolite_name>Spectral Match to Ser-Val from NIST14</metabolite_name><metabolite_name>(1r,3r)-1-Aminocyclopentane-1,3-dicarboxylic acid</metabolite_name><metabolite_name>N-Valyltryptophan</metabolite_name><metabolite_name>Serine</metabolite_name><metabolite_name>8,12-Octadecadienoic acid</metabolite_name><metabolite_name>4-Methylbenzaldehyde</metabolite_name><metabolite_name>Glutathione (oxidized)</metabolite_name><metabolite_name>Leucyl-alanine</metabolite_name><metabolite_name>Isocitric acid</metabolite_name><metabolite_name>Dibutylamine</metabolite_name><metabolite_name>N,N-Dimethyl-N-decylamine</metabolite_name><metabolite_name>Furanone A</metabolite_name><metabolite_name>Non-3-enoylcarnitine</metabolite_name><metabolite_name>LPC(18:1)</metabolite_name><metabolite_name>5-(2H-benzo[3,4-d]1,3-dioxolen-5-ylcarbonylamino)-2-(methylamino)-1,3-thiazole -4-carboxamide</metabolite_name><metabolite_name>Tridec-8-enoylcarnitine</metabolite_name><metabolite_name>5-Acetoxyindole</metabolite_name><metabolite_name>Prostaglandin B1</metabolite_name><metabolite_name>2-Hydroxypurine</metabolite_name><metabolite_name>Methylene-imidazole</metabolite_name><metabolite_name>1-Pentyl-3-(1-naphthoyl) indole</metabolite_name><metabolite_name>methyl 1-methylpiperidine-3-carboxylate</metabolite_name><metabolite_name>Spectral Match to Arachidonoylthio-PC from NIST14</metabolite_name><metabolite_name>3-(beta-D-Glucopyranosyloxy)-7-(alpha-L-rhamnopyranosyloxy)-5-hydroxy-4'-methoxyflavone</metabolite_name><metabolite_name>Stearic acid amide</metabolite_name><metabolite_name>Fructosyl-lysine</metabolite_name><metabolite_name>Dodecanamide</metabolite_name><metabolite_name>ethyl 2-[(ethoxycarbonyl)carbonylamino]benzoate</metabolite_name><metabolite_name>Pseudo-anisatin</metabolite_name><metabolite_name>Valylvaline</metabolite_name><metabolite_name>4,1-Benzoxazepine</metabolite_name><metabolite_name>4-Aminobutyraldehyde</metabolite_name><metabolite_name>6beta-Hydroxycortisol</metabolite_name><metabolite_name>Propionyl-L-carnitine</metabolite_name><metabolite_name>Sphingosine</metabolite_name><metabolite_name>Glyceryl monostearate</metabolite_name><metabolite_name>Malonylcarnitine</metabolite_name><metabolite_name>1-Lauroyl-2-Hydroxy-sn-Glycero-3-Phosphocholine</metabolite_name><metabolite_name>gamma-Glutamylleucine</metabolite_name><metabolite_name>gamma-Glutamylmethionine</metabolite_name><metabolite_name>1H-Pyrrole-2-carboxaldehyde</metabolite_name><metabolite_name>AgelasineE/F</metabolite_name><metabolite_name>L-IsoleucineL-Isoleucine</metabolite_name><metabolite_name>L,L-Cyclo(prolylalanyl)</metabolite_name><metabolite_name>L-Cystathionine</metabolite_name><metabolite_name>Lysine</metabolite_name><metabolite_name>Isoleucylserine</metabolite_name><metabolite_name>AICAR</metabolite_name><metabolite_name>Tyrosylleucine</metabolite_name><metabolite_name>2-Pyrrolecarboxylic acid</metabolite_name><metabolite_name>Guanine</metabolite_name><metabolite_name>17Alpha-hydroxy progesterone</metabolite_name><metabolite_name>4-[5-[[4-[5-[Acetyl(hydroxy)amino]pentylamino]-4-oxobutanoyl]-hydroxyamino]pentylamino]-4-oxobutanoic acid</metabolite_name><metabolite_name>Nepsilon,nepsilon,nepsilon-trimethyllysine</metabolite_name><metabolite_name>Dehydrocostus lactone</metabolite_name><metabolite_name>N-Acetylphenylalanine</metabolite_name><metabolite_name>Xanthosine</metabolite_name><metabolite_name>Perseitol</metabolite_name><metabolite_name>3,4-Diaminopyridine</metabolite_name><metabolite_name>Citric acid</metabolite_name><metabolite_name>Glycylleucine</metabolite_name><metabolite_name>Retinol</metabolite_name><metabolite_name>Isospaglumic acid</metabolite_name><metabolite_name>3-Formyl-6-hydroxyindole</metabolite_name><metabolite_name>(2E,4E)-Octadeca-2,4-dienoylcarnitine</metabolite_name><metabolite_name>N-Acetyl-L-arginine</metabolite_name><metabolite_name>Spectral Match to Ile-Gly-Ile from NIST14</metabolite_name><metabolite_name>3-Hydroxybenzyl alcohol</metabolite_name><metabolite_name>NAD+</metabolite_name><metabolite_name>Desogestrel</metabolite_name><metabolite_name>2,6,10,14-Hexadecatetraenoic acid, 3,7,11,15-tetramethyl-, (Z,E,E)-</metabolite_name><metabolite_name>Benzeneacetonitrile</metabolite_name><metabolite_name>(aS,5S)-Metolachlor</metabolite_name><metabolite_name>Leucyl-Glutamine</metabolite_name><metabolite_name>Spectral Match to Thr-Leu from NIST14</metabolite_name><metabolite_name>Indole-3-acrylic acid</metabolite_name><metabolite_name>C9H8O2</metabolite_name><metabolite_name>Phenylalanyltryptophan</metabolite_name><metabolite_name>3,4,5-Trimethylbenzaldehyde</metabolite_name><metabolite_name>2-Aminoethyl oleate</metabolite_name><metabolite_name>Glutathione (reduced)</metabolite_name><metabolite_name>2-(2-Aminopropanylamino)-4-carbamoylbutyric acid</metabolite_name><metabolite_name>5,5-Dimethyl-2(5H)-furanone</metabolite_name><metabolite_name>4-[(2,4-Dihydroxy-3,3-dimethylbutanoyl)amino]butanoic acid</metabolite_name><metabolite_name>6-Hydroxynicotinic acid</metabolite_name><metabolite_name>5-hydroxycalamenene</metabolite_name><metabolite_name>5-Hydroxymaltol</metabolite_name><metabolite_name>2-Pyrazinoylguanidine</metabolite_name><metabolite_name>Anandamide</metabolite_name><metabolite_name>Hydroquinone</metabolite_name><metabolite_name>Bisphenol A</metabolite_name><metabolite_name>Tryptophan methyl ester</metabolite_name><metabolite_name>Benzofuran</metabolite_name><metabolite_name>Spectral Match to 15-HEDE from NIST14</metabolite_name><metabolite_name>4-Hexanolide</metabolite_name><metabolite_name>1,2-Dihydroquinoline</metabolite_name><metabolite_name>Prostaglandin I3</metabolite_name><metabolite_name>dihydrocytochalasin B</metabolite_name><metabolite_name>3',4',5',5,7-Pentamethoxyflavone</metabolite_name><metabolite_name>15-Methylheptadecanoylcarnitine</metabolite_name><metabolite_name>Threonyltryptophan</metabolite_name><metabolite_name>Protoanemonin</metabolite_name><metabolite_name>1-Myristoyl-2-palmitoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>3-Methylnonanedioylcarnitine</metabolite_name><metabolite_name>N-6-Isopent-2-enyl-adenosine</metabolite_name><metabolite_name>2-(4-Methyl-1,3-thiazol-5-yl)ethanol</metabolite_name><metabolite_name>4-Hydroxybenzyl alcohol</metabolite_name><metabolite_name>N-Acetylasparagine</metabolite_name><metabolite_name>Vinyl carbamate</metabolite_name><metabolite_name>Eicosanoids_12,13diHOME_C18H34O4</metabolite_name><metabolite_name>L-3-Aminodihydro-2(3H)-furanone</metabolite_name><metabolite_name>1-Methyladenosine</metabolite_name><metabolite_name>1-(2-Hydroxyethyl)-2,2,6,6-tetramethyl-4-hydroxypiperidine</metabolite_name><metabolite_name>Avenoleic acid</metabolite_name><metabolite_name>Sarcosine</metabolite_name><metabolite_name>Prolylvaline</metabolite_name><metabolite_name>Methyl arachidonate</metabolite_name><metabolite_name>Heptanoylcarnitine</metabolite_name><metabolite_name>7,8-Dihydro-D-biopterin</metabolite_name><metabolite_name>16-Hydroxypalmitic acid</metabolite_name><metabolite_name>Taurine</metabolite_name><metabolite_name>Zymosterol</metabolite_name><metabolite_name>2'-CMP</metabolite_name><metabolite_name>Arachidic acid</metabolite_name><metabolite_name>Spectral Match to Phe-Gln from NIST14</metabolite_name><metabolite_name>Pirbuterol</metabolite_name><metabolite_name>Spectral Match to Maltotetraose from NIST14</metabolite_name><metabolite_name>Epigallocatechol</metabolite_name><metabolite_name>Dexmethylphenidate</metabolite_name><metabolite_name>Pyridoxal</metabolite_name><metabolite_name>Trigonelline</metabolite_name><metabolite_name>beta-Methylcrotonylglycine</metabolite_name><metabolite_name>N-Acetyl-D-glucosamine</metabolite_name><metabolite_name>Catechol</metabolite_name><metabolite_name>1,10-Epoxygermacrone</metabolite_name><metabolite_name>Spectral Match to Ile-Glu-Lys from NIST14</metabolite_name><metabolite_name>Spectral Match to Val-Ile from NIST14</metabolite_name><metabolite_name>D-gamma-Glutamyl-D-glutamic acid</metabolite_name><metabolite_name>Dihydroceramide</metabolite_name><metabolite_name>Butenylcarnitine</metabolite_name><metabolite_name>1,2,3,4,Tetrahydro-1,5,7-trimethylnapthalene</metabolite_name><metabolite_name>4-Methylproline</metabolite_name><metabolite_name>2-(4-methoxyphenyl)propan-2-ol</metabolite_name><metabolite_name>(5Z,8R,9S,11R,13E,15S,19R)-9,11,15,19-Tetrahydroxy-5,13-prostadienoic acid</metabolite_name><metabolite_name>gamma-Guanidinobutyric acid</metabolite_name><metabolite_name>4-Ethylaniline</metabolite_name><metabolite_name>(2-{[(2R)-2-hydroxy-3-(pentadecanoyloxy)propyl phosphonato]oxy}ethyl)trimethylazanium</metabolite_name><metabolite_name>7-Methylnonanoylcarnitine</metabolite_name><metabolite_name>4-Hydroxymethylpyrazole</metabolite_name><metabolite_name>N-Hydroxy-1-aminonaphthalene</metabolite_name><metabolite_name>4,6-Cholestadien-3-one</metabolite_name><metabolite_name>Rishitinol</metabolite_name><metabolite_name>Nonactinic acid</metabolite_name><metabolite_name>Glycocyamine</metabolite_name><metabolite_name>Phenylalanine</metabolite_name><metabolite_name>Piperonyl butoxide</metabolite_name><metabolite_name>2-(2-amino-3-methylpentanamido)-3-carbamoylpropanoic acid</metabolite_name><metabolite_name>Tamoxifen</metabolite_name><metabolite_name>Cholesterol</metabolite_name><metabolite_name>Cholestenone</metabolite_name><metabolite_name>L-beta-Homoleucine</metabolite_name><metabolite_name>Gentiatibetine</metabolite_name><metabolite_name>Pro-leu</metabolite_name><metabolite_name>Morpholine</metabolite_name><metabolite_name>Moluccanin</metabolite_name><metabolite_name>Parthenolide</metabolite_name><metabolite_name>6,7-dimethoxyisoquinolyl 4,5-dimethoxy-2-nitrophenyl ketone</metabolite_name><metabolite_name>3-Methoxy-4-hydroxyphenylethanol</metabolite_name><metabolite_name>3-Hydroxy-2H-pyran-2-one</metabolite_name><metabolite_name>Spectral Match to Lys-Leu from NIST14</metabolite_name><metabolite_name>Myosmine</metabolite_name><metabolite_name>Uracil</metabolite_name><metabolite_name>Vidarabine</metabolite_name><metabolite_name>2,6-Di-tert-butyl-4-hydroxymethylphenol</metabolite_name><metabolite_name>3-Methylbenzaldehyde</metabolite_name><metabolite_name>(3S,8aS)-3-Isobutylhexahydropyrrolo[1,2-a]pyrazine-1,4-dione</metabolite_name><metabolite_name>beta-D-Glucopyranoside, 2-(hydroxymethyl)phenyl</metabolite_name><metabolite_name>Creatine</metabolite_name><metabolite_name>4-Oxo-L-proline</metabolite_name><metabolite_name>Sphingosine 1-phosphate</metabolite_name><metabolite_name>2-Hydroxyphenylethanol</metabolite_name><metabolite_name>Octopine</metabolite_name><metabolite_name>Spectral Match to Valerylcarnitine from NIST14</metabolite_name><metabolite_name>Fenpropimorph-carboxy</metabolite_name><metabolite_name>beta-Maltose</metabolite_name><metabolite_name>hydroxydeoxyguanosine</metabolite_name><metabolite_name>Methyl galactoside</metabolite_name><metabolite_name>2-Aminobicyclo[3.1.0]hexane-2,6-dicarboxylic acid</metabolite_name><metabolite_name>Phenylalanyl-Alanine</metabolite_name><metabolite_name>2-(2-amino-3-phenylpropanamido)-3-methylbutanoic acid</metabolite_name><metabolite_name>2-Ethyl-3-hydroxy-2,4,4-trimethyloxazolidine</metabolite_name><metabolite_name>Guanidinosuccinic acid</metabolite_name><metabolite_name>Maltoxazine</metabolite_name><metabolite_name>Simazine</metabolite_name><metabolite_name>3-(2-Hydroxy-3-methoxyphenyl)-2-propenoic acid</metabolite_name><metabolite_name>Acadesine</metabolite_name><metabolite_name>GABA</metabolite_name><metabolite_name>Hept-4-enoylcarnitine</metabolite_name><metabolite_name>Adrenic acid</metabolite_name><metabolite_name>(8Z,11Z,13E,15S)-15-hydroxyicosa-8,11,13-trienoic acid</metabolite_name><metabolite_name>Benzothiazolone</metabolite_name><metabolite_name>Desmosterol</metabolite_name><metabolite_name>3h-Taurine</metabolite_name><metabolite_name>Threoninyl-gamma-glutamate</metabolite_name><metabolite_name>1,3,5-Hexatriene</metabolite_name><metabolite_name>cis-Dihydrocarvone</metabolite_name><metabolite_name>Spectral Match to Ser-Leu from NIST14</metabolite_name><metabolite_name>Glycylmethionine</metabolite_name><metabolite_name>2-Hydroxy-4-imino-2,5-cyclohexadienone</metabolite_name><metabolite_name>2,3-Dihydro-1H-pyrrolizine-5-carboxaldehyde</metabolite_name><metabolite_name>Thunberginol A</metabolite_name><metabolite_name>Pyrrolidine-2-carboxamide</metabolite_name><metabolite_name>Deoxycarnitine</metabolite_name><metabolite_name>(5Z,8Z,11Z)-13-{3-[(2Z)-pent-2-en-1-yl]oxiran-2-yl}trideca-5,8,11-trienoic acid</metabolite_name><metabolite_name>3beta,6beta-Dihydroxynortropane</metabolite_name><metabolite_name>3-Octadec-9-enoyloxy-4-(trimethylazaniumyl)butanoate</metabolite_name><metabolite_name>(9E,12E)-N-(2-Hydroxyethyl)octadeca-9,12-dienamide</metabolite_name><metabolite_name>3-Aminobenzamide</metabolite_name><metabolite_name>trans-Hexadec-2-enoyl carnitine</metabolite_name><metabolite_name>3-(Hydroxymethyl)furan-2-carbaldehyde</metabolite_name><metabolite_name>4',6-Dihydroxyflavone</metabolite_name><metabolite_name>C9H14N</metabolite_name><metabolite_name>1,2-Dimethylimidazole</metabolite_name><metabolite_name>Threonine, DL-</metabolite_name><metabolite_name>Dihydro-5-methyl-2(3H)-furanone</metabolite_name><metabolite_name>Dimethylethanolamine</metabolite_name><metabolite_name>3-(Octyloxy)propanamine</metabolite_name><metabolite_name>(1S,2R,4aR,8aR)-1-Acetoxy-7-isopropylidene-1,4a-dimethyl-6-oxodecahydro-2-naphthalenyl 2,3-dimethyl-2-oxiranecarboxylate</metabolite_name><metabolite_name>Histidine</metabolite_name><metabolite_name>cis-5-Tetradecenoylcarnitine</metabolite_name><metabolite_name>7Z,10Z-Hexadecadienoic acid</metabolite_name><metabolite_name>Tropic acid</metabolite_name><metabolite_name>4-Hydroxy-2H-pyran-3-carboxaldehyde</metabolite_name><metabolite_name>Glutamic acid gamma-methyl ester</metabolite_name><metabolite_name>Phosphoric acid</metabolite_name><metabolite_name>(9E)-Heptadec-9-enoylcarnitine</metabolite_name><metabolite_name>12-Hydroxydodecanoic acid</metabolite_name><metabolite_name>Pyridin-2-ol</metabolite_name><metabolite_name>3, 5-Tetradecadiencarnitine</metabolite_name><metabolite_name>N-Phenylacetylglutamic acid</metabolite_name><metabolite_name>2-Hydroxylauroylcarnitine</metabolite_name><metabolite_name>Cysteinylglycine</metabolite_name><metabolite_name>Tyrosine methyl ester</metabolite_name><metabolite_name>Diethyl phthalate</metabolite_name><metabolite_name>Glyceryl monopalmitate</metabolite_name><metabolite_name>Ergothioneine</metabolite_name><metabolite_name>Undec-3-enoylcarnitine</metabolite_name><metabolite_name>Medrysone</metabolite_name><metabolite_name>(2'E,4'Z,8E)-Colneleic acid</metabolite_name><metabolite_name>Quinoline-4-carboxylic acid</metabolite_name><metabolite_name>(4Z,7Z,10Z,13Z,16Z)-18-(3-ethyloxiran-2-yl)octadeca-4,7,10,13,16-pentaenoic acid</metabolite_name><metabolite_name>Histidiylleucine</metabolite_name><metabolite_name>1-Dodecyl-2-pyrrolidinone</metabolite_name><metabolite_name>Kynurenine</metabolite_name><metabolite_name>2-(2-aminopropanamido)-4-methylpentanoic acid</metabolite_name><metabolite_name>8-hydroxy-5Z,9E,11Z,14Z-eicosatetraenoic acid</metabolite_name><metabolite_name>N-Acetylhistidine</metabolite_name><metabolite_name>6-Bromoquinolin-2(1H)-one</metabolite_name><metabolite_name>3-(2-(2H,3H-benzo[3,4-e]1,4-dioxin-6-yl)-2-oxoethyl)-3-hydroquinazolin-4-one</metabolite_name><metabolite_name>3-Pyridinecarboxaldehyde</metabolite_name><metabolite_name>Tryptophan</metabolite_name><metabolite_name>Xanthine</metabolite_name><metabolite_name>Polyalanine</metabolite_name><metabolite_name>Choline</metabolite_name><metabolite_name>Dodeca-2,4,6-trienoylcarnitine</metabolite_name><metabolite_name>Trp-Pro</metabolite_name><metabolite_name>2-Hexenoylcarnitine</metabolite_name><metabolite_name>2-Hydroxyacetamide</metabolite_name><metabolite_name>N-Butylbenzenesulfonamide</metabolite_name><metabolite_name>Capryloyl glycine</metabolite_name><metabolite_name>1H-Imidazole-4-propanol, beta-amino-, (S)-</metabolite_name><metabolite_name>9,11-Octadecadienoic acid, 13-oxo-, (E,Z)-</metabolite_name><metabolite_name>L-Isoleucine</metabolite_name><metabolite_name>6-Oxopiperidine-2-carboxylic acid</metabolite_name><metabolite_name>Vitispirane</metabolite_name><metabolite_name>S-lactoyl-glutathione</metabolite_name><metabolite_name>Trimethyl glycine</metabolite_name><metabolite_name>2-Aminopurine</metabolite_name><metabolite_name>(6E,9E,12E)-Hexadeca-6,9,12-trienoylcarnitine</metabolite_name><metabolite_name>3-(Tetradecanoylamino)propyl(carboxymethyl)dimethylammonium</metabolite_name><metabolite_name>Palmitoylcarnitine</metabolite_name><metabolite_name>Phosphorylcholine</metabolite_name><metabolite_name>IMP</metabolite_name><metabolite_name>S-Adenosylhomocysteine</metabolite_name><metabolite_name>Transtorine</metabolite_name><metabolite_name>2,2,6,6-Tetramethylpiperidin-1-ol</metabolite_name><metabolite_name>D-Lysine</metabolite_name><metabolite_name>Fasoracetam</metabolite_name><metabolite_name>Dimethyl sulfoxide</metabolite_name><metabolite_name>L-Aspartic acid</metabolite_name><metabolite_name>Spectral Match to .alpha.-L-Glu-L-Tyr from NIST14</metabolite_name><metabolite_name>Unknown (carbon number 9)</metabolite_name><metabolite_name>9-Octadecenamide</metabolite_name><metabolite_name>Uridine monophosphate</metabolite_name><metabolite_name>Echimidine N-oxide</metabolite_name><metabolite_name>Phthalic anhydride</metabolite_name><metabolite_name>3-Hydroxybutyrylcarnitine</metabolite_name><metabolite_name>(R)-alpha-Methylhistamine</metabolite_name><metabolite_name>1-Palmitoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>Ethoxyquin</metabolite_name><metabolite_name>N-acetylglucosamine</metabolite_name><metabolite_name>(2-aminoethoxy)[(2R)-2-hydroxy-3-[(9Z)-octadec-9-enoyloxy]propoxy]phosphinic acid</metabolite_name><metabolite_name>Acetyl glutamic acid</metabolite_name><metabolite_name>racemic-Propranolol</metabolite_name><metabolite_name>2-Acetyl-1,5,6,7-tetrahydro-6-hydroxy-7-(hydroxymethyl)-4H-azepine-4-one</metabolite_name><metabolite_name>Caffeoylcholine</metabolite_name><metabolite_name>Pyridoxal phosphate</metabolite_name><metabolite_name>Indole-3-carboxylic acid</metabolite_name><metabolite_name>3-Hydroxy-cis-5-tetradecenoylcarnitine</metabolite_name><metabolite_name>Phosphoric acid, diphenyl ester</metabolite_name><metabolite_name>4-oxo-Butanal</metabolite_name><metabolite_name>Glycyl-L-phenylalanine</metabolite_name><metabolite_name>alpha-Methyltryptamine</metabolite_name><metabolite_name>Riboflavin</metabolite_name><metabolite_name>N-Isovaleroylglycine</metabolite_name><metabolite_name>Alanylnorvaline</metabolite_name><metabolite_name>Palmidrol</metabolite_name><metabolite_name>1-(Aminocarbonyl)-L-proline</metabolite_name><metabolite_name>Trimethyloxamine</metabolite_name><metabolite_name>19-Hydroxydeoxycorticosterone</metabolite_name><metabolite_name>Semidehydroascorbic acid</metabolite_name><metabolite_name>N-Phenyl-1-naphthylamine</metabolite_name><metabolite_name>7-Oxodehydroepiandrosterone 3-acetate</metabolite_name><metabolite_name>Cholestan-3-one</metabolite_name><metabolite_name>m-Aminobenzoic acid</metabolite_name><metabolite_name>Biotin</metabolite_name><metabolite_name>GMP</metabolite_name><metabolite_name>Phenol</metabolite_name><metabolite_name>Spectral Match to Asp-Leu from NIST14</metabolite_name><metabolite_name>2-Furanmethanol</metabolite_name><metabolite_name>(2-{[(2R)-2-hydroxy-3-(tetradecanoyloxy)propyl phosphonato]oxy}ethyl)trimethylazanium</metabolite_name><metabolite_name>3-Azetidinecarboxylic acid</metabolite_name><metabolite_name>L-Acetylleucine</metabolite_name><metabolite_name>Dicyclohexylamine</metabolite_name><metabolite_name>Cinnamic acid</metabolite_name><metabolite_name>4-(2-Furanylmethylene)-3,4-dihydro-2H-pyrrole</metabolite_name><metabolite_name>Butyrylcarnitine</metabolite_name><metabolite_name>Spectral Match to 12(S)-Hydroxy-16-heptadecynoic acid from NIST14</metabolite_name><metabolite_name>Aspartyl adenylate</metabolite_name><metabolite_name>Spectral Match to Ser-Phe from NIST14</metabolite_name><metabolite_name>4'-Hydroxyflavanone</metabolite_name><metabolite_name>Spectral Match to Monopalmitolein (9c) from NIST14</metabolite_name><metabolite_name>cis-4-Carboxymethylenebut-2-en-4-olide</metabolite_name><metabolite_name>1-Piperidinecarboxaldehyde</metabolite_name><metabolite_name>(2E)-2-Propylpent-2-enoylcarnitine</metabolite_name><metabolite_name>Benzododecinium</metabolite_name><metabolite_name>3-Methylpentanoylcarnitine</metabolite_name><metabolite_name>N(6)-(N-Threonylcarbonyl)adenosine</metabolite_name><metabolite_name>Hydroxyindoleacetic acid</metabolite_name><metabolite_name>3-(2-Methylpropyl)pyridine</metabolite_name><metabolite_name>(7E)-5-Hydroxydodec-7-enoylcarnitine</metabolite_name><metabolite_name>Irigenin Trimethyl Ether</metabolite_name><metabolite_name>2-[2-amino-3-(4-hydroxyphenyl)propanamido]-3-phenylpropanoic acid</metabolite_name><metabolite_name>dihydrocorticosterone</metabolite_name><metabolite_name>Isoferulic acid</metabolite_name><metabolite_name>4',5,6,7-Tetramethoxyflavone</metabolite_name><metabolite_name>9-Decenoylcarnitine</metabolite_name><metabolite_name>Octadeca-9,12-dienal</metabolite_name><metabolite_name>3-Hydroxyisovalerylcarnitine</metabolite_name><metabolite_name>2-(2-amino-3-carbamoylpropanamido)-3-phenylpropanoic acid</metabolite_name><metabolite_name>Tiglylcarnitine</metabolite_name><metabolite_name>Tridecanoylcarnitine</metabolite_name><metabolite_name>Aspartyl-phenylalanine</metabolite_name><metabolite_name>3-Hydroxyhexadecanoylcarnitine</metabolite_name><metabolite_name>Thiamine ion</metabolite_name><metabolite_name>3-oxo-Butanal</metabolite_name><metabolite_name>Guanosine</metabolite_name><metabolite_name>Spectral Match to 8S-Hydroxy-9E,11Z,14Z-eicosatrienoic acid from NIST14</metabolite_name><metabolite_name>Pregn-4-en-18-al, 11,21-dihydroxy-3,20-dioxo-, (11.beta.)-</metabolite_name><metabolite_name>1-acyl-PAF</metabolite_name><metabolite_name>gamma-Glutamylisoleucine</metabolite_name><metabolite_name>beta-D-Glucosamine</metabolite_name><metabolite_name>Methyl DL-methionate</metabolite_name><metabolite_name>5-Butyl-2-methylpyridine</metabolite_name><metabolite_name>Isoindolin-1-one</metabolite_name><metabolite_name>25-Hydroxycholesterol</metabolite_name><metabolite_name>2-Pyrrolidone</metabolite_name><metabolite_name>1,2-Dipalmitoleoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>Tris(1,3-dichloro-2-propyl)phosphate</metabolite_name><metabolite_name>Spectral Match to Leu-Ile from NIST14</metabolite_name><metabolite_name>Alanylphenylalanine</metabolite_name><metabolite_name>2,6 Dimethylheptanoyl carnitine</metabolite_name><metabolite_name>(2E,6E,8E)-Decatrienoylcarnitine</metabolite_name><metabolite_name>Bullatine G</metabolite_name><metabolite_name>4-methylquinoline-2-carboxylic acid</metabolite_name><metabolite_name>Uridine</metabolite_name><metabolite_name>4-Amino-2-methylenebutanoic acid</metabolite_name><metabolite_name>Acetyl proline</metabolite_name><metabolite_name>1-Carboxyethylphenylalanine</metabolite_name><metabolite_name>6,10,14-Trimethyl-5,9,13-pentadecatrien-2-one</metabolite_name><metabolite_name>2,2,7,7-Tetramethyl-1,6-dioxaspiro[4.4]nona-3,8-diene</metabolite_name><metabolite_name>Nicotinamide mononucleotide</metabolite_name><metabolite_name>Unknown (carbon number 11)</metabolite_name><metabolite_name>3-acetyl-5-(butan-2-yl)-4-hydroxy-2,5-dihydro-1H-pyrrol-2-one</metabolite_name><metabolite_name>3-hydroxydecanoyl carnitine</metabolite_name><metabolite_name>Equol</metabolite_name><metabolite_name>(1s,4r)-7,7-dimethyl-2-oxobicyclo[2.2.1]heptane-1-carboxylic acid</metabolite_name><metabolite_name>Proline</metabolite_name><metabolite_name>3-Methyl-4-phenyl-3-buten-2-one</metabolite_name><metabolite_name>Tetrahydropyridine</metabolite_name><metabolite_name>Macrosphelide D</metabolite_name><metabolite_name>8-Dodecenoylcarnitine</metabolite_name><metabolite_name>Isoindoline</metabolite_name><metabolite_name>Spectral Match to Ile-Leu from NIST14</metabolite_name><metabolite_name>N6-Succinyladenosine</metabolite_name><metabolite_name>5,7-Dihydroxy-4-methylcoumarin</metabolite_name><metabolite_name>Capric dimethyl amine oxide</metabolite_name><metabolite_name>Indole-3-acetic acid</metabolite_name><metabolite_name>Tinnevellin glucoside</metabolite_name><metabolite_name>Isovalerylglutamic acid</metabolite_name><metabolite_name>Tetradecyldiethanolamine</metabolite_name><metabolite_name>6,15-Diketo-13,14-dihydroprostaglandin F1alpha</metabolite_name><metabolite_name>Fumaric acid</metabolite_name><metabolite_name>Acetylcarnitine</metabolite_name><metabolite_name>Uric Acid</metabolite_name><metabolite_name>Argininosuccinic acid</metabolite_name><metabolite_name>2-Methylthio-N6-(delta2-isopentenyl)adenosine</metabolite_name><metabolite_name>Hypotaurine</metabolite_name><metabolite_name>(2R)-6-methylpiperidine-2-carboxylic acid</metabolite_name><metabolite_name>Sucrose</metabolite_name><metabolite_name>Phenylalanylphenylalanine</metabolite_name><metabolite_name>Viridicatin</metabolite_name><metabolite_name>gamma-Glutamyltyrosine</metabolite_name><metabolite_name>Isoguanosine</metabolite_name><metabolite_name>LPC(22:6)</metabolite_name><metabolite_name>Glycylproline</metabolite_name><metabolite_name>Mmv688888</metabolite_name><metabolite_name>Spermidine</metabolite_name><metabolite_name>Taurocholic acid</metabolite_name><metabolite_name>Spectral Match to Tyr-Val from NIST14</metabolite_name><metabolite_name>Propionylcarnitine</metabolite_name><metabolite_name>Dioctyl phthalate</metabolite_name><metabolite_name>Octa-2,6-dienoylcarnitine</metabolite_name><metabolite_name>20-Hydroxy-5,8,11,14-eicosatetraenoic acid</metabolite_name><metabolite_name>3-(Dodecanoylamino)propyl(carboxymethyl)dimethylammonium</metabolite_name><metabolite_name>5-Hydroxy-4-oxo-L-norvaline</metabolite_name><metabolite_name>2H-benzo[d]1,3-dioxolan-5-yl-N-(4,5-dimethyl(1,3-thiazol-2-yl))carboxamide</metabolite_name><metabolite_name>Acetone cyanohydrin</metabolite_name><metabolite_name>Glutamic acid</metabolite_name><metabolite_name>Psicose</metabolite_name><metabolite_name>Suberic acid</metabolite_name><metabolite_name>Hydroxyproline</metabolite_name><metabolite_name>1H-Imidazole-4-carboxamide</metabolite_name><metabolite_name>4-Hexylaniline</metabolite_name><metabolite_name>2-(5-methyl-1,3-dioxobenzo[c]azolin-2-yl)acetic acid</metabolite_name><metabolite_name>5'-Methylthioadenosine</metabolite_name><metabolite_name>Hydroxyacetone</metabolite_name><metabolite_name>delta-Valerobetaine</metabolite_name><metabolite_name>Formamide, N-[(1Z)-2-(4-methoxyphenyl)ethenyl]-</metabolite_name><metabolite_name>Creatinine</metabolite_name><metabolite_name>Dexpanthenol</metabolite_name><metabolite_name>3,4,5-trimethoxyphenyl 3,4,5-trimethylpyrazolyl ketone</metabolite_name><metabolite_name>Serylalanine</metabolite_name><metabolite_name>4-Vinylcyclohexene</metabolite_name><metabolite_name>Pyridoxic acid</metabolite_name><metabolite_name>Pyridoxine</metabolite_name><metabolite_name>1-O-Hexadecyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>Di-N-propylamine</metabolite_name><metabolite_name>7,8-Dimethylalloxazine</metabolite_name><metabolite_name>3-Hydroxyhexanoylcarnitine</metabolite_name><metabolite_name>N-acetylgalactosamine</metabolite_name><metabolite_name>N-L-Alanyl-L-valine</metabolite_name><metabolite_name>9-Tetradecenoic acid</metabolite_name><metabolite_name>Clupanodonic acid</metabolite_name><metabolite_name>Triethyl phosphate</metabolite_name><metabolite_name>3-Methylene-indolenine</metabolite_name><metabolite_name>Cortisol</metabolite_name><metabolite_name>Cycloserine</metabolite_name><metabolite_name>Propamocarb</metabolite_name><metabolite_name>Hydroxy Tyrosol -Acetate</metabolite_name><metabolite_name>methyl (7Z)-hexadec-7-enoate</metabolite_name><metabolite_name>Urobilin</metabolite_name><metabolite_name>Betaine</metabolite_name><metabolite_name>Pivaloylcarnitine</metabolite_name><metabolite_name>(2-aminoethoxy)[(2R)-3-(hexadecanoyloxy)-2-hydroxypropoxy]phosphinic acid</metabolite_name><metabolite_name>Metacetamol</metabolite_name><metabolite_name>Cyclopentanone</metabolite_name><metabolite_name>1-Aminocyclopropane-1-carboxylic acid</metabolite_name><metabolite_name>4-({4-[methyl(methylsulfonyl)amino]phenoxy}methyl)hydroquinolin-2-one</metabolite_name><metabolite_name>Cytidine</metabolite_name><metabolite_name>Taurursodiol</metabolite_name><metabolite_name>N-Acetyl-L-aspartic acid</metabolite_name><metabolite_name>(R)-2-Nitro-p-mentha-1,5-diene</metabolite_name><metabolite_name>3-Hydroxytetradeca-6,8,10-trienoylcarnitine</metabolite_name><metabolite_name>Bethanidine</metabolite_name><metabolite_name>Indole-3-lactic acid</metabolite_name><metabolite_name>5,6-Dihydroxy-2-methylaminotetralin</metabolite_name><metabolite_name>C17 Sphingosine</metabolite_name><metabolite_name>Norethindrone</metabolite_name><metabolite_name>Sphinganine</metabolite_name><metabolite_name>Glycerophosphoethanolamine</metabolite_name><metabolite_name>Mead acid</metabolite_name><metabolite_name>1-Pyrrolidinecarboxaldehyde</metabolite_name><metabolite_name>7-Ethoxycoumarin</metabolite_name><metabolite_name>1-Methyl-L-tryptophan</metabolite_name><metabolite_name>7-Ketolithocholic acid</metabolite_name><metabolite_name>m-Ethyl_toluene</metabolite_name><metabolite_name>1H-Indene-1,2(3H)-dione</metabolite_name><metabolite_name>(1S,2S,6R,7R,9R)-6-methyl-10,12-dioxatricyclo[7.2.1.0&lt;2,7>]dodec-4-en-8-one</metabolite_name><metabolite_name>Indole-3-carbaldehyde</metabolite_name><metabolite_name>Progesterone</metabolite_name><metabolite_name>3-Ethylpyridine</metabolite_name><metabolite_name>Ricinoleic acid, methyl ester</metabolite_name><metabolite_name>6-oxo-Butanal</metabolite_name><metabolite_name>Isohexadecanoic acid</metabolite_name><metabolite_name>Cyclopenta[c]pyran-4-carboxylic acid, 1-(beta-D-glucopyranosyloxy)-1,4a,5,7a-tetrahydro-7-[[[(2E)-1-oxo-3-phenyl-2-propen-1-yl]oxy]methyl]-, (1S,4aS,7aS)-</metabolite_name><metabolite_name>Bistris</metabolite_name><metabolite_name>Spectral Match to O-Arachidonoylglycidol from NIST14</metabolite_name><metabolite_name>N-Caproylglycine</metabolite_name><metabolite_name>Kynurenic acid</metabolite_name><metabolite_name>beta-Alanine</metabolite_name><metabolite_name>Noradrenaline</metabolite_name><metabolite_name>Norleucine, (+/-)-</metabolite_name><metabolite_name>Prolylisoleucine</metabolite_name><metabolite_name>1,2-Benzoquinone</metabolite_name><metabolite_name>Phenylalanylleucine</metabolite_name><metabolite_name>Thiamine monophosphate</metabolite_name><metabolite_name>2-Acetylacteoside</metabolite_name><metabolite_name>Methionine</metabolite_name><metabolite_name>Muramic acid</metabolite_name><metabolite_name>Gibberellin A12</metabolite_name><metabolite_name>Sinapine</metabolite_name><metabolite_name>Isoleucyl-Arginine</metabolite_name><metabolite_name>Styrene Oxide</metabolite_name><metabolite_name>Niacin</metabolite_name><metabolite_name>Lauroylcarnitine</metabolite_name><metabolite_name>3-Ethylfuran</metabolite_name><metabolite_name>2-Methyl-2-phenoxypropanoic acid</metabolite_name><metabolite_name>PC(18:1(11Z)/18:2(9Z,12Z))</metabolite_name><metabolite_name>(+/-)-8-hydroxy-5Z,9E,11Z,14Z,17Z-eicosapentaenoic acid</metabolite_name><metabolite_name>Spectral Match to Met-Val from NIST14</metabolite_name><metabolite_name>1H-Purine</metabolite_name><metabolite_name>Tris(chloroethyl) phosphate</metabolite_name><metabolite_name>(5E,7E)-Dodeca-5,7-dienoylcarnitine</metabolite_name><metabolite_name>Spectral Match to Ile-Pro from NIST14</metabolite_name><metabolite_name>Carnosine</metabolite_name><metabolite_name>8-HETrE</metabolite_name><metabolite_name>Lauryldiethanolamine</metabolite_name><metabolite_name>3-Methoxytyramine</metabolite_name><metabolite_name>Palmitoyl glycerol</metabolite_name><metabolite_name>N-[1-(4-methoxy-6-oxopyran-2-yl)-2-methylpropyl]acetamide</metabolite_name><metabolite_name>Mmv688469</metabolite_name><metabolite_name>(1R,2R,4aS,8aS)-2-[(2R)-2-Hydroxybutyl]-1,3-dimethyl-1,2,4a,5,6,7,8,8a-octahydro-1-naphthalenecarboxylic acid</metabolite_name><metabolite_name>1,2-Dilinolenoyl-sn-glycero-3-phosphatidylcholine</metabolite_name><metabolite_name>N-Fructosyl pyroglutamate</metabolite_name><metabolite_name>Niacinamide</metabolite_name><metabolite_name>1-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>Serotonin</metabolite_name><metabolite_name>6-Keto-decanoylcarnitine</metabolite_name><metabolite_name>Kojic acid</metabolite_name><metabolite_name>2'-Deoxyguanosine 5'-phosphate</metabolite_name><metabolite_name>Phenylacetylglycine</metabolite_name><metabolite_name>2-(3-amino-6-iminoxanthen-9-yl)benzoic acid, chloride</metabolite_name><metabolite_name>Methyl sterculate</metabolite_name><metabolite_name>(2E)-Hexenedioylcarnitine</metabolite_name><metabolite_name>Spectral Match to Ile-Tyr from NIST14</metabolite_name><metabolite_name>1-Octadecanoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>1,2,3,4-Tetrahydro-beta-carboline-3-carboxylic acid</metabolite_name><metabolite_name>Nadide</metabolite_name><metabolite_name>1-Piperideine-2-carboxylic acid</metabolite_name><metabolite_name>1-Phenyl-1-cyclohexene</metabolite_name><metabolite_name>6-Oxopurine</metabolite_name><metabolite_name>5-(4-carboxy-3-methylbutyl)-5,6,8a-trimethyl-3-oxo-4a,6,7,8-tetrahydro-4H-naphthalene-1-carboxylic acid</metabolite_name><metabolite_name>Spectral Match to Leu-Glu from NIST14</metabolite_name><metabolite_name>Erythrocentaurin</metabolite_name><metabolite_name>Threonylleucine</metabolite_name><metabolite_name>Phytosphingosine</metabolite_name><metabolite_name>Isoleucylhydroxyproline</metabolite_name><metabolite_name>Spectral Match to Glu-Phe from NIST14</metabolite_name><metabolite_name>Spectral Match to 4(5)-EpDPE methyl ester from NIST14</metabolite_name><metabolite_name>Tyrosine</metabolite_name><metabolite_name>Gallic acid</metabolite_name><metabolite_name>15-Deoxy-delta12,14-prostaglandin J2</metabolite_name><metabolite_name>Glyceryl monooleate</metabolite_name><metabolite_name>5,8,11,14-Eicosatetraenoic acid, ethyl ester</metabolite_name><metabolite_name>5-Methylpyrazine-2-carboxylic Acid</metabolite_name><metabolite_name>Lauramine oxide</metabolite_name><metabolite_name>Imidazolone</metabolite_name><metabolite_name>4-Methylimidazole</metabolite_name><metabolite_name>Spermine</metabolite_name><metabolite_name>alpha-Eleostearic acid</metabolite_name><metabolite_name>4-carbamoyl-2-acetamidobutanoic acid</metabolite_name><metabolite_name>Hypoxanthine</metabolite_name><metabolite_name>9-acetyl-17-hydroxy-10,14-dimethyl-20-oxahexacyclo[11.9.0.0&lt;2,10>.0&lt;4,9>.0&lt;14, 19>.0&lt;19,21>]docosane</metabolite_name><metabolite_name>Hippuric acid</metabolite_name><metabolite_name>Linarin</metabolite_name><metabolite_name>Spectral Match to Val-Phe from NIST14</metabolite_name><metabolite_name>D-Pipecolic acid</metabolite_name><metabolite_name>4-Deoxypyridoxine</metabolite_name><metabolite_name>3-Amino-2,2-dimethylpropanoic acid</metabolite_name><metabolite_name>6,7-Dihydroxy-4-methylcoumarin</metabolite_name><metabolite_name>2-Ethylpropanedioylcarnitine</metabolite_name><metabolite_name>Spectral Match to .alpha.-L-Asp-L-Phe from NIST14</metabolite_name><metabolite_name>Negletein</metabolite_name><metabolite_name>Ophthalmic acid</metabolite_name><metabolite_name>N-Acetyl-beta-neuraminic acid</metabolite_name><metabolite_name>4-Methylheptanoylcarnitine</metabolite_name><metabolite_name>Adenine</metabolite_name><metabolite_name>FAD</metabolite_name><metabolite_name>8-Methyltridecanoylcarnitine</metabolite_name><metabolite_name>Nicotinuric acid</metabolite_name><metabolite_name>Timonacic</metabolite_name><metabolite_name>Inosine</metabolite_name><metabolite_name>Spectral Match to Arachidonoyl amide from NIST14</metabolite_name><metabolite_name>Leu-leu-ome</metabolite_name><metabolite_name>6-Hydroxy-3,4-dihydro-2(1H)-quinolinone</metabolite_name><metabolite_name>Carbapenem</metabolite_name><metabolite_name>Unknown (carbon number 16)</metabolite_name><metabolite_name>Metolachlor-Morpholinone</metabolite_name><metabolite_name>Diprotin A</metabolite_name><metabolite_name>Cholic acid</metabolite_name><metabolite_name>m-Toluamide</metabolite_name><metabolite_name>Arachidonic acid</metabolite_name><metabolite_name>Pregnenolone</metabolite_name><metabolite_name>Arachidonoyl-carnitine</metabolite_name><metabolite_name>3-Hydroxy-5,8-tetradecadienoylcarnitine</metabolite_name><metabolite_name>Ellagic acid</metabolite_name><metabolite_name>Anserine</metabolite_name><metabolite_name>3-Methylhistidine</metabolite_name><metabolite_name>Caffeine</metabolite_name><metabolite_name>4-Hydroxybenzoylcholine</metabolite_name><metabolite_name>1-Palmitoyl-2-oleoyl-sn-glycero-3-phosphocholine</metabolite_name><metabolite_name>PC(16:0/22:5(4Z,7Z,10Z,13Z,16Z))</metabolite_name><metabolite_name>Chebi</metabolite_name><metabolite_name>Glyceryl monolinoleate</metabolite_name><metabolite_name>gamma-Glutamylcysteine</metabolite_name><metabolite_name>L-Methionine</metabolite_name><metabolite_name>Dihydrouracil</metabolite_name><metabolite_name>3-Hydroxy-4-oxo-4H-pyran-2-carboxylic acid</metabolite_name><metabolite_name>8-Methylquinoline</metabolite_name><metabolite_name>alpha-Methylene butyrolactone</metabolite_name><metabolite_name>Pro-val</metabolite_name><metabolite_name>Dimethyl phthalate</metabolite_name><metabolite_name>Tri(2-butoxyethyl) phosphate</metabolite_name><metabolite_name>Glyceryl monomyristate</metabolite_name><metabolite_name>Mannitol</metabolite_name><metabolite_name>sn-Glycero-3-phosphocholine</metabolite_name><metabolite_name>Glucose 1-phosphate</metabolite_name><metabolite_name>Tri-(2-chloroisopropyl)phosphate</metabolite_name><metabolite_name>(15Z)-tetracos-15-enoylcarnitine</metabolite_name><metabolite_name>Octanoic acid, 2-amino-</metabolite_name><metabolite_name>Polyglutamine</metabolite_name><metabolite_name>Succinylcarnitine</metabolite_name><metabolite_name>Dodecanedioic acid</metabolite_name><metabolite_name>(7R)-7-[(Dodeca-4,7,10-trienoyl)oxy]-4-hydroxy-N,N,N-trimethyl-4-oxo-3,5,9-trioxa-4lambda~5~-phosphapentacosan-1-aminium</metabolite_name><metabolite_name>(3E,5Z)-1,3,5-Heptatriene</metabolite_name><metabolite_name>Dimethyl myristamine</metabolite_name><metabolite_name>Indane</metabolite_name><metabolite_name>Tetradeca-5,7,9-trienoylcarnitine</metabolite_name><metabolite_name>N-Lauroylsarcosine</metabolite_name><metabolite_name>Dibutyl phthalate</metabolite_name><metabolite_name>4-(2-Aminophenyl)-2,4-dioxobutanoic acid</metabolite_name><metabolite_name>(1R,5R,6R,13R,14R,16S)-6-(3-Furyl)-16-(2-methoxy-2-oxoethyl)-1,5,15,15-tetramethyl-8,17-dioxo-7-oxatetracyclo[11.3.1.0~2,11~.0~5,10~]heptadec-10-en-14-yl 2-methylpropanoate</metabolite_name><metabolite_name>3-Methylglutarylcarnitine</metabolite_name><metabolite_name>Methylpyrazine</metabolite_name><metabolite_name>Methylmalonic acid</metabolite_name><metabolite_name>8-Oxo-7,8-dihydrodeoxyguanine</metabolite_name><metabolite_name>2-(6-Aminopurin-9-yl)-5-(hydroxymethyl)oxolane-3,4-diol</metabolite_name><metabolite_name>Spectral Match to 13(S)-HODE methyl ester from NIST14</metabolite_name><metabolite_name>Malic acid</metabolite_name><metabolite_name>Spectral Match to Trp-Glu from NIST14</metabolite_name><metabolite_name>1-Carboxyethyltyrosine</metabolite_name><metabolite_name>3-ethyl-4-hydroxy-1-benzylhydroquinolin-2-one</metabolite_name><metabolite_name>Oxolinic acid</metabolite_name><metabolite_name>Indole-3-acetaldehyde</metabolite_name><metabolite_name>Vitamin K1</metabolite_name><metabolite_name>Erucamide</metabolite_name><metabolite_name>Dimethyl-3-hydroxypyrid-4-one</metabolite_name><metabolite_name>Bufalin</metabolite_name><metabolite_name>3-Aminopicolinaldehyde</metabolite_name><metabolite_name>Oxypurinol</metabolite_name><metabolite_name>2-Benzimidazolecarbamic acid</metabolite_name><metabolite_name>Coronaric acid</metabolite_name><metabolite_name>Traversianal</metabolite_name><metabolite_name>Denudatine</metabolite_name><metabolite_name>3-hydroxyoctanoyl carnitine</metabolite_name><metabolite_name>(8S,9S,10R,11S,13S,14S,17S)-11,17-dihydroxy-17-(2-hydroxyacetyl)-10,13-dimethyl-2,6,7,8,9,11,12,14,15,16-decahydro-1H-cyclopenta[a]phenanthren-3-one</metabolite_name><metabolite_name>3-(Indol-3-yl)propionic acid</metabolite_name><metabolite_name>6,11-dioxododecanoic acid</metabolite_name><metabolite_name>Methionine sulfoxide</metabolite_name><metabolite_name>Normetanephrine</metabolite_name><metabolite_name>6,8-Dihydroxypurine</metabolite_name><metabolite_name>1,3-Dicyclohexylurea</metabolite_name><metabolite_name>Hexadecanamide</metabolite_name><metabolite_name>trimethyl(2-{[(2R)-3-[(9Z)-octadec-9-enoyloxy]-2-(tetradecanoyloxy)propyl phosphonato]oxy}ethyl)azanium</metabolite_name><metabolite_name>Gly-Tyr</metabolite_name><metabolite_name>Threonylphenylalanine</metabolite_name><metabolite_name>p-Hydroxybenzaldehyde</metabolite_name><metabolite_name>Azelaic acid</metabolite_name><metabolite_name>2-(2-amino-3-methylpentanamido)-3-hydroxybutanoic acid</metabolite_name><metabolite_name>Spectral Match to Leu-Trp from NIST14</metabolite_name><metabolite_name>Spectral Match to 3-Hydroxyoctadecanoic Acid from NIST14</metabolite_name><metabolite_name>Triphenyl phosphate</metabolite_name><metabolite_name>Imidazolepropionic acid</metabolite_name><metabolite_name>Epinephrine</metabolite_name><metabolite_name>2-(Methylsulfonyl)pyridine</metabolite_name><metabolite_name>2-Methyl-1,4-benzoquinone</metabolite_name><metabolite_name>4,5-Dimethyloxazole</metabolite_name><metabolite_name>SM(d18:0/18:1(11Z))</metabolite_name><metabolite_name>5?-CHOLANIC ACID-3?-OL-6_7-DIONE</metabolite_name><metabolite_name>Spectral Match to Ile-Met from NIST14</metabolite_name><metabolite_name>2-trans,4-cis-Decadienoylcarnitine</metabolite_name><metabolite_name>(1S,5R,9S,13R)-14-formyl-5,9-dimethyltetracyclo[11.2.1.0,?.0?,?]hexadec-14-ene-5-carboxylic acid</metabolite_name><metabolite_name>N,N-Dimethyl-1-tetradecanamine-N-oxide</metabolite_name><metabolite_name>Daidzein</metabolite_name><metabolite_name>SM(d18:2(4E,14Z)/16:0)</metabolite_name><metabolite_name>3-Hydroxyadipic acid 3,6-lactone</metabolite_name><metabolite_name>N-epsilon-Acetyllysine</metabolite_name><metabolite_name>(2-{[(2R)-3-(heptadecanoyloxy)-2-hydroxypropyl phosphonato]oxy}ethyl)trimethylazanium</metabolite_name></additional><is_claimable>false</is_claimable><name>Genetic Ablation of Sfxn5 Induces Mitochondrial Dysfunction and Precipitates Lethal Metabolic Crisis in Mice</name><description>Mitochondrial disorders frequently manifest with life-threatening hepatic metabolic crises. Using a global Sfxn5-knockout mouse model, we investigated the systemic consequences of disrupting this mitochondrial inner-membrane transporter through integrated biochemical, metabolomic, histological, and ultrastructural analyses. Sfxn5 deficiency resulted in complete postnatal lethality accompanied by severe metabolic collapse and progressive multi-organ dysfunction. The liver emerged as the primary site of pathology, exhibiting marked mitochondrial structural damage and widespread disruption of central metabolic pathways, including the tricarboxylic acid cycle, fatty acid β-oxidation, and ammonia detoxification, leading to hyperammonemia and systemic metabolic stress. Importantly, liver-specific reconstitution of Sfxn5 partially restored mitochondrial metabolic function, substantially reduced hyperammonemia, and alleviated multi-organ pathology. Altogether, these findings identify hepatic mitochondrial dysfunction as the central driver of Sfxn5-dependent lethality and establish a critical role for Sfxn5 in maintaining mitochondrial metabolic homeostasis during early postnatal 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