{"database":"MassIVE","file_versions":[{"headers":{"Content-Type":["application/json"]},"body":{"files":{"Other":["ftp://massive-ftp.ucsd.edu/v08/MSV000095020/"]},"type":"primary"},"statusCode":"OK","statusCodeValue":200}],"scores":null,"additional":{"omics_type":["Proteomics"],"submitter":["Jesus Mateos"],"instrument_platform":["ZenoTOF 7600"],"species":["Homo Sapiens (ncbitaxon:9606)"],"full_dataset_link":["https://massive.ucsd.edu/ProteoSAFe/dataset.jsp?task=5df720b803b44455be9556b914f0f1fd"],"submitter_email":["jesusmateosmartin@gmail.com"],"submitter_affiliation":["Spanish Research Council CSIC"],"sample_protocol":[""],"repository":["MassIVE"],"file_size":["256"],"ptm_modification":["ALKYKATION Carbamidomethyl on cysteine C2H3NO [C] +57Da"],"data_protocol":[""],"name_synonyms":["2-amino-, CDF, Mercamine, beta Mercaptoethylamine, 35S Labeled, determination, Mercaptoethylamine, Desmoplastic astrocytoma of infancy, Cysteamine Tartrate, CG1768, DRF2, Cysteamine Tartrate (1:1), ms(2)04138, Desmoplastic infantile astrocytoma, Cysteamine Dihydrochloride, POF, 2-Aminoethanethiol, Tosylate, 35S-Labeled, DmelCG1768, MLPLI, chemical analysis, HILDA, Hydrochloride, AGAMOUS-like 61, Dias, Hydrobromide, Cysteinamine, DiA, Mercaptamine, Cysteamine Hydrobromide, Cysteamine Bitartrate, l(2)k07135, Cysteamine, POF2, F27C12_24, Cysteamine Hydrochloride, Cysteamine Maleate (1:1), Tartrate, F27C12.24, Bitartrate, 2 Aminoethanethiol, Ethanethiol, DIASP, DIA, Dia, Fibroblast., 4-(4-dihexadecylaminostyryl)N-methylpyridium iodide, Dihydrochloride, Becaptan, Cysteamine Tosylate, DIANA, 38E.16, beta-Mercaptoethylamine, Cystagon, assay, 35S-Labeled Cysteamine, DIA2"],"description_synonyms":["Delayed Treatments, Forms, \"cystine storage disease\" EXACT [CSP2005:1849-0607], 0998/12, chronic renal failure, lifespan, Defect of, 35S Labeled, Cystine Diseases, End-Stage Renal Failure, Diatheses, Mercaptoethylamine, PNT-P1, ETS2, Ets2, DmelCG17077, Cystinoses, EY3-1, Pnt, Cystine Storage Diseases, side effects, End-Stage Kidney Disease, Cysteamine Tartrate (1:1), Renal Disease, DMPOINT1A, pntegfr, Defect Of, Life Quality, 0608/07, png, 35S-Labeled, End stage renal disease, Line, symptoms, End Stage Kidney Disease, 3, Chronic Kidney Failure, D-ets-2, 3520, Hydrobromide, Chronic Renal Failure, \"Cystine storage disease\" EXACT [SNOMEDCT_2005_07_31:190683000], Health Related Quality Of Life, Pnt-P1, Orphan Disease, ESRD, treatment, Cystine Diathesis, Mercaptamine, Cysteamine Hydrobromide, Cysteamine Bitartrate, 3'-dithiobis(2-aminopropanoic acid), pointed-RC, entire lifespan, Cystine Storage Disease, cystine diathesis, entire life cycle, Cystine, Cysteamine Maleate (1:1), Bitartrate, Zystin, Ethanethiol, Storage Disease, Health-Related Quality Of Life, primary dermal fibroblast, EK3-2, l(3)07825, Nephropathic Cystinoses, Dihydrochloride, chronic renal failure syndrome, Clients, disease management, Therapies, \"Cystinosis (disorder) [Ambiguous]\" EXACT [SNOMEDCT_2005_07_31:111398009], Chronic, CG17077, Copper Cystinate, End-stage renal failure, End stage renal failure, l(3)j1B7, \"Cystinosis [dup] (disorder)\" EXACT [SNOMEDCT_2005_07_31:367374009], 2-amino-, Therapy, screening, Delayed Treatment, Nephropathic, Disease, Ets, Mercamine, findings, beta Mercaptoethylamine, Diathesis, L Cystine, mouse embryonic fibroblast, process quality of life cycle, alpha-Diamino-beta-dithiolactic acid, Delay, Cystinosis, Renal Failure, Cell Lines, D-Ets-2, Cysteamine Tartrate, \"Cystinosis (disorder)\" EXACT [SNOMEDCT_2005_07_31:190681003], ets94F, 0123/09, Client, Cell, Dicysteine, Cystinosin, L-Cystine, Cysteamine Dihydrochloride, disease management., 2-Aminoethanethiol, Tosylate, Treatment Delays, end-stage renal disease, cystine disease, Hydrochloride, Cystine Disease, HRQOL, Cysteinamine, pnt-P1, Delays, pnt-P2, End Stage, l(3)s118306, Lines, Rare Disease, Cystine Diatheses, pntP2, Cysteamine, Drives, Pointed-P1, Kidney Disease, life, Cysteamine Hydrochloride, Tartrate, Ets94F, signs, 2 Aminoethanethiol, Lysosomal Cystine Transport Protein, Nephropathic Cystinosis, Treatments, Orphan, ptd, End-Stage Renal, Delayed, PntP2, Rare, Ets58AB, Therapeutic, Patient, Becaptan, PntP1, E(E2F)3D, Cysteamine Tosylate, Defect of Cystinosin, Cystin, beta-Mercaptoethylamine, Cystagon, Storage Diseases, Treatment, End-Stage Renal Disease, PNTP2, 35S-Labeled Cysteamine, cistina, End-Stage, End Stage Renal Disease, End-Stage Kidney, Orphan Diseases, PNTP1, POINT, Fibroblast, CG8705"],"additional_accession":[]},"is_claimable":false,"name":"DIA-SWATH analysis of the effect of cysteamine in cystinotic fibroblasts","description":"Fibroblast cell lines form cystinotic and non-cystinotic fibroblast were incubated for 48h with increasing amounts of cysteamine, the only available treatment for cystinosis, an incurable metabolic rare disease that is characterized by accumulation of cystine crystals in the cells and accompanied by detrimental symptoms at the renal, ocular, and muscular levels. Cysteamine treatment delays the symptoms and improves the quality of life of the patients, but the patients depend on it for life. Furthermore, oral cysteamine treatment present undesirable side effects and fails in avoiding the end-stage renal failure that inevitably drives to kidney transplant. Our results will be helpful to understand, from a molecular point of view, the benefits, deficiencies, and detrimental effects of the cysteamine treatment.","dates":{"publication":"Thu Jun 13 05:51:00 BST 2024"},"accession":"MSV000095020","cross_references":{}}