Seroatlas · Human Serome Atlas

COASY

Bifunctional coenzyme A synthase

Also known as: COASY_HUMAN, DPCK, NBP, PPAT

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
Q13057
Gene
COASY
Ensembl
ENSG00000068120
Chromosome
17
Canonical length
564 aa
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted intracellular proteins
Subcellular location
Mitochondria,Connecting piece
Secretome location
Intracellular and membrane

OverviewNCBI Gene

Coenzyme A (CoA) functions as a carrier of acetyl and acyl groups in cells and thus plays an important role in numerous synthetic and degradative metabolic pathways in all organisms. In eukaryotes, CoA and its derivatives are also involved in membrane trafficking and signal transduction. This gene encodes the bifunctional protein coenzyme A synthase (CoAsy) which carries out the last two steps in the biosynthesis of CoA from pantothenic acid (vitamin B5). The phosphopantetheine adenylyltransferase domain of this bifunctional protein catalyzes the conversion of 4'-phosphopantetheine into dephospho-coenzyme A (dpCoA) while its dephospho-CoA kinase domain completes the final step by phosphorylating dpCoA to form CoA. Mutations in this gene are associated with neurodegeneration with brain iron accumulation (NBIA). Alternative splicing results in multiple isoforms. [provided by RefSeq, Apr 2014]

Canonical amino-acid sequenceUniProt

564 residues, UniProt reviewed canonical sequence.

>Q13057|COASY
     1  MAVFRSGLLV LTTPLASLAP RLASILTSAA RLVNHTLYVH LQPGMSLEGP AQPQSSPVQA
    61  TFEVLDFITH LYAGADVHRH LDVRILLTNI RTKSTFLPPL PTSVQNLAHP PEVVLTDFQT
   121  LDGSQYNPVK QQLVRYATSC YSCCPRLASV LLYSDYGIGE VPVEPLDVPL PSTIRPASPV
   181  AGSPKQPVRG YYRGAVGGTF DRLHNAHKVL LSVACILAQE QLVVGVADKD LLKSKLLPEL
   241  LQPYTERVEH LSEFLVDIKP SLTFDVIPLL DPYGPAGSDP SLEFLVVSEE TYRGGMAINR
   301  FRLENDLEEL ALYQIQLLKD LRHTENEEDK VSSSSFRQRM LGNLLRPPYE RPELPTCLYV
   361  IGLTGISGSG KSSIAQRLKG LGAFVIDSDH LGHRAYAPGG PAYQPVVEAF GTDILHKDGI
   421  INRKVLGSRV FGNKKQLKIL TDIMWPIIAK LAREEMDRAV AEGKRVCVID AAVLLEAGWQ
   481  NLVHEVWTAV IPETEAVRRI VERDGLSEAA AQSRLQSQMS GQQLVEQSHV VLSTLWEPHI
   541  TQRQVEKAWA LLQKRIPKTH QALD

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against COASY can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Other membrane
Secreted
No
Transmembrane segments
0
Mean surface accessibility (rSASA)
0.26
Highest tissue expression
66 nTPM

Expression across tissuesHPA

Tissue

  • liver: 66 nTPM
  • adrenal gland: 56 nTPM
  • pancreas: 55 nTPM
  • salivary gland: 55 nTPM
  • esophagus: 51 nTPM
  • kidney: 48 nTPM

Single-cell type

  • hepatocytes: 115 nCPM
  • cytotrophoblasts: 106 nCPM
  • breast lactating cells: 105 nCPM
  • syncytiotrophoblasts: 95 nCPM
  • esophageal suprabasal cells: 85 nCPM
  • migrating cytotrophoblasts: 79 nCPM

Immune cell

  • myeloid DC: 34 nTPM
  • intermediate monocyte: 32 nTPM
  • classical monocyte: 30 nTPM
  • eosinophil: 27 nTPM
  • total PBMC: 24 nTPM
  • plasmacytoid DC: 24 nTPM

Brain region

  • pons: 37 nTPM
  • choroid plexus: 34 nTPM
  • cerebral cortex: 33 nTPM
  • thalamus: 33 nTPM
  • medulla oblongata: 32 nTPM
  • hippocampal formation: 31 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about COASY.

Disease | AllUniProt

Conditions COASY is implicated in, by any mechanism.

  • Neurodegeneration with brain iron accumulation 6 (NBIA6) MIM:615643
  • Pontocerebellar hypoplasia 12 (PCH12) MIM:618266

Disease | GeneticClinVar

31 pathogenic / likely-pathogenic of 411 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)
1.03
gnomAD pLI
0
gnomAD missense Z
0.11
DepMap mean gene effect
-0.49
DepMap dependency class
selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 6% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of COASY in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads COASY as an antibody target. Whether an autoantibody or antibody against COASY could matter depends on whether native COASY is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

COASY is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label COASY as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/COASY. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

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