Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Analysis of neutrophil proteome of Barth syndrome patients

ABSTRACT: Barth syndrome (BTHS) is a rare genetic disease caused by mutations in the TAFAZZIN gene. It is characterized by neutropenia, cardiomyopathy, and skeletal myopathy. Neutropenia in BTHS is associated with life-threatening infections, yet the molecular and physiological causes of this phenomenon are poorly understood. We conducted proteome analysis of circulating neutrophils from BTHS patients to identify upregulated and downregulated canonical pathways that may explain the disruptions in neutrophil maturation and function.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Neutrophil, Blood

DISEASE(S): Barth Syndrome

SUBMITTER: Borko Amulic

LAB HEAD: Borko Amulic

PROVIDER: PXD052714 | Pride | 2025-02-12

REPOSITORIES: Pride

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
	040719-10Plex-TMT-PS.msf	Msf
	040719-10Plex-TMT.pdResult	Other
	Fraction-1.raw	Raw
	Fraction-10.raw	Raw
	Fraction-11.raw	Raw

Items per page:

1 - 5 of 17

Similar Datasets

Enhancement of red blood cell transfusion compatibility using CRISPR-mediated erythroblast gene editing

Project description:Regular blood transfusion is the cornerstone of care for patients with red blood cell (RBC) disorders such as thalassemia or sickle cell disease. With repeated transfusion, alloimmunisation often occurs due to incompatibility at the level of minor blood group antigens. We use CRISPR-mediated genome editing of an immortalised human erythroblast cell line (BEL-A) to generate multiple enucleation competent cell lines deficient in individual blood groups. Edits are combined to generate a single cell line deficient in multiple antigens responsible for the most common transfusion incompatibilities: ABO (Bombay phenotype), Rh (Rhnull), Kell (K0), Duffy (Duffynull), GPB (S- s- U-). These cells can be differentiated to generate deformable reticulocytes, illustrating the capacity for coexistence of multiple rare blood group antigen null phenotypes. This study provides the first proof-of-principle demonstration of combinatorial CRISPR-mediated blood group gene editing to generate customisable or multi-compatible RBCs for diagnostic reagents or recipients with complicated matching requirements.

2018-04-26 | PXD009291 | Pride

Molecular identification of a BAR domain-containing coat complex for endosomal recycling of transmembrane proteins

Project description:Protein trafficking requires coat complexes that couple recognition of sorting motifs in transmembrane cargos with biogenesis of transport carriers. The mechanisms of cargo transport through the endosomal network are poorly understood. Here, we identify a sorting motif for endosomal recycling of cargos including the cation-independent mannose-6-phosphate receptor and semaphorin 4C by the membrane tubulating BAR domain-containing sorting nexins SNX5 and SNX6. Crystal structures establish that this motif folds into a β-hairpin that binds a site in the SNX5/SNX6 phox homology domains. Over sixty cargos share this motif and require SNX5/SNX6 for their recycling. These include cargos involved in neuronal migration and a Drosophila snx6 mutant displays defects in axonal guidance. These studies identify a sorting motif and provide molecular insight into an evolutionary conserved coat complex, the ‘Endosomal SNX-BAR sorting complex for promoting exit 1’ (ESCPE-1), which couples sorting motif recognition to BAR domain-mediated biogenesis of cargo-enriched tubulo-vesicular transport carriers.

2021-05-28 | PXD014927 | Pride

Comparative LC-MSMS Analysis of Extracellular Vesicles Extracted from Mouse Caecum in Fasted vs. Fed Mice

Project description:Bacterial extracellular vesicles (BEVs) produced by members of the intestinal microbiota can contribute not only to digestion but also mediate microbe-host cell communication via the transfer of functional biomolecules to mammalian host cells. An unresolved question is what host factors and conditions influence BEV cargoes and how do they impact on host cell function? To address this question, we analysed and compared the proteome of BEVs released by the major human gastrointestinal tract (GIT) symbiont Bacteroides thetaiotaomicron (Bt) in vivo in fed versus fasted animals using nano-liquid chromatography with tandem mass spectrometry (LC-MSMS). Among the proteins whose abundance was negatively affected by fasting, nine of ten proteins of the serine protease family, including the regulatory protein dipeptidyl peptidase-4 (DPP-4), were significantly decreased in BEVs produced in the GIT of fasted animals. Strikingly, in ex-tracellular vesicles produced by the intestinal epithelium of the same fasted mice, the proteins with the most increased abundance were serine protease inhibitors (serpins). Together, these findings suggest a dynamic interaction between GI bacteria and the host. Additionally, they indicate a regulatory role for the host in determining the balance be-tween bacterial serine proteases and host serpins exported in bacterial and host extra-cellular vesicles.

2025-05-26 | PXD062737 | Pride

Mia3 enables efficient secretion by constraining COPII vesicle budding - secreted proteome

Project description:Analysis of secreted proteome from cells engineered with knockout of Mia3 gene isoforms encoding TANGO1L and TANGO1S

2021-08-25 | PXD024221 | Pride

Placental macrophage (Hofbauer cell) response to viral and bacterial ligands is influenced by fetal sex

Project description:Bacterial and viral infections of the placenta are associated with inflammation and adverse pregnancy outcomes. Hofbauer cells (HBCs) are specialised fetal-origin macrophages in the placental villi and are proposed to protect the fetus from vertical transmission of pathogens; however, they are poorly understood. Here, we have performed quantitative proteomics on term HBCs under resting conditions and following exposure to bacterial and viral pathogen associated molecular patterns (PAMPs), and investigated the contribution of fetal sex to these responses. Resting HBCs expressed a plethora of proteins pertinent to macrophage function, including chemokines, cytokines, Toll-like receptors, and classical and non-classical major histocompatibility complex class I and II molecules. HBCs mounted divergent responses to bacterial versus viral PAMPs but exhibited protein expression changes suggestive of a switch towards a more pro-inflammatory phenotype. A comparison between male and female HBCs, showed that the latter mounted a much stronger and wider response. Sexual dimorphism in HBCs was primarily associated with lipid metabolism in males and cytoskeleton organisation in females. We provide a novel and comprehensive understanding regarding the phenotype of term placental macrophages and their sex-dependent responses to infectious triggers.

2022-10-31 | PXD033006 | Pride

Characterisation of proteome of a novel Escherichia coli strain exhibiting mucoviscous phenotype.

Project description:Protein expression by E. coli 26561 during the late-exponential phase of cultures under anaerobic conditions was examined. E. coli 26561 is a multidrug resistant (MDR) and shows an unusual hyper-mucoviscous phenotype. Resistance includes ESBL (CTX-M-14) and proteome was determined with and without exposure to sub-MIC concentrations of the 3rd generation cephalosporin ceftazidime. Ceftazidime exposure was at two sub-MIC levels, specifically 0.25x MIC (samples 5-7), 0.5x MIC (samples 8 - 10); samples 1-4 provided the unexposed Control. Both whole and phospho-enriched fractions for each sample were analysed. Quantification of peptides was assessed using 10-plex TMT labelling in conjunction with an Orbitrap Fusion Tribrid. Raw data produced by the Orbitrap were processed using Max Quant 1.5.4.7 using the included Andromeda search engine. Peptides were searched against our own database of E. coli 26561 proteins which was produced from a hybrid assembly of our reads obtained from MiSeq and PacBio sequencing platforms.

2018-08-22 | PXD008339 | Pride

Identification of a VPS29 isoform with restricted association to Retriever and Retromer accessory proteins through autoinhibition

Project description:The endosomal-lysosomal network is a hub of organelles that orchestrate the dynamic sorting of hundreds of integral membrane proteins to maintain cellular homeostasis. VPS29 is a central conductor of this network through its assembly into Retromer, Retriever and Commander endosomal sorting complexes, and its role in regulating RAB GTPase activity. Two VPS29 isoforms have been described, VPS29A and VPS29B, that differ solely in their amino-terminal sequences. Here we identify a third VPS29 isoform, which we term VPS29C, that harbours an extended amino-terminal sequence compared to VPS29A and VPS29B. Through a combination of AlphaFold predictive modelling, in vitro complex reconstitution, mass spectrometry and molecular cell biology, we find that the amino-terminal VPS29C extension constitutes an autoinhibitory sequence that limits access to a hydrophobic groove necessary for effector protein recruitment to Retromer, and association with Retriever and Commander. VPS29C is therefore unique in its ability to uncouple Retromer-dependent cargo sorting from the broader roles of VPS29A and VPS29B in regulating the endosomal-lysosomal network through accessory protein recruitment. Our identification and characterisation of VPS29C points to additional complexity in the differential subunit assembly of Retromer, an important consideration given the increasing interest in Retromer as a potential therapeutic target in neurodegenerative diseases.

2025-07-21 | PXD064959 | Pride

Respiratory Health Outcomes in Neonates (RHiNO) Study Urine Proteomics

Project description:Urine sampled from preterm-born (<34 weeks gestation) school-aged (7-12yrs) children and term born (>/=37 weeks gestation). Spirometry, exercise testing and cardiovascular assessment performed at time of sampling. Preter-born children with low lung function entered a randomised placebo controlled trial of inhaler therapies.

2023-07-31 | PXD042759 | Pride

ChAdOx infection of human cell lines A549 MRC-5

Project description:Total proteome and phospho proteome of two human cell lines infected with the ChAdOx1 nCoV-19 virus over 72 hours

2021-01-11 | PXD023286 | Pride

Chemical degradation of BTK/TEC as a novel approach to inhibit platelet function.

Project description:Determination of the effect of the generic tyrosine kinase degrader TL12-186, and the BTK degraders DD-04-15 and DD-03-171 on human platelet protein content and function.

2023-03-02 | PXD034440 | Pride

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data