MitoProteome Database

MT001357

Record overview

MITO ID	MT001357
Gene ID	80347
Species	Homo sapiens (Human)
Gene Name	CoA synthase
Gene Symbol	COASY
Synonyms	NBP; DPCK; PPAT; UKR1; NBIA6; pOV-2;
Alternate names	bifunctional coenzyme A synthase; nucleotide binding protein; phosphopantetheine adenylyltransferase / dephosphocoenzyme A kinase; bifunctional phosphopantetheine adenylyl transferase/dephospho CoA kinase;
Chromosome	17
Map Location	17q12-q21
EC Number	2.7.7.3
Summary	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]
Orthologs	View orthologs and multiple alignments for COASY

Proteins

bifunctional coenzyme A synthase isoform a precursor

Refseq ID:	NP_001035994
Protein GI:	110227601
UniProt ID:	Q13057
mRNA ID:	NM_001042529
Length:	564
RefSeq Status:

MAVFRSGLLVLTTPLASLAPRLASILTSAARLVNHTLYVHLQPGMSLEGPAQPQSSPVQATFEVLDFITHLYAGADVHRHLDVRILLTNIRTKSTFLPPL
PTSVQNLAHPPEVVLTDFQTLDGSQYNPVKQQLVRYATSCYSCCPRLASVLLYSDYGIGEVPVEPLDVPLPSTIRPASPVAGSPKQPVRGYYRGAVGGTF
DRLHNAHKVLLSVACILAQEQLVVGVADKDLLKSKLLPELLQPYTERVEHLSEFLVDIKPSLTFDVIPLLDPYGPAGSDPSLEFLVVSEETYRGGMAINR
FRLENDLEELALYQIQLLKDLRHTENEEDKVSSSSFRQRMLGNLLRPPYERPELPTCLYVIGLTGISGSGKSSIAQRLKGLGAFVIDSDHLGHRAYAPGG
PAYQPVVEAFGTDILHKDGIINRKVLGSRVFGNKKQLKILTDIMWPIIAKLAREEMDRAVAEGKRVCVIDAAVLLEAGWQNLVHEVWTAVIPETEAVRRI
VERDGLSEAAAQSRLQSQMSGQQLVEQSHVVLSTLWEPHITQRQVEKAWALLQKRIPKTHQALD

bifunctional coenzyme A synthase isoform a precursor
`N sig_peptide: 1..23 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2398 peptide sequence: MAVFRSGLLVLTTPLASLAPRLA`

Refseq ID:	NP_079509
Protein GI:	110227603
UniProt ID:	Q13057
mRNA ID:	NM_025233
Length:	564
RefSeq Status:

Protein sequence is identical to GI:110227601 (mRNA isoform)

N
sig_peptide: 1..23
inference: COORDINATES: ab initio prediction:SignalP:4.0
calculated_mol_wt: 2398
peptide sequence: 
MAVFRSGLLVLTTPLASLAPRLA

sig_peptide: 1..23
inference: COORDINATES: ab initio prediction:SignalP:4.0
calculated_mol_wt: 2398
peptide sequence: 
MAVFRSGLLVLTTPLASLAPRLA

bifunctional coenzyme A synthase isoform c

Refseq ID:	NP_001035997
Protein GI:	205277394
UniProt ID:	Q13057
mRNA ID:	NM_001042532
Length:	593
RefSeq Status:

MRTPRLRAQPRGAVYQAPSPPPAPVGLGSMAVFRSGLLVLTTPLASLAPRLASILTSAARLVNHTLYVHLQPGMSLEGPAQPQSSPVQATFEVLDFITHL
YAGADVHRHLDVRILLTNIRTKSTFLPPLPTSVQNLAHPPEVVLTDFQTLDGSQYNPVKQQLVRYATSCYSCCPRLASVLLYSDYGIGEVPVEPLDVPLP
STIRPASPVAGSPKQPVRGYYRGAVGGTFDRLHNAHKVLLSVACILAQEQLVVGVADKDLLKSKLLPELLQPYTERVEHLSEFLVDIKPSLTFDVIPLLD
PYGPAGSDPSLEFLVVSEETYRGGMAINRFRLENDLEELALYQIQLLKDLRHTENEEDKVSSSSFRQRMLGNLLRPPYERPELPTCLYVIGLTGISGSGK
SSIAQRLKGLGAFVIDSDHLGHRAYAPGGPAYQPVVEAFGTDILHKDGIINRKVLGSRVFGNKKQLKILTDIMWPIIAKLAREEMDRAVAEGKRVCVIDA
AVLLEAGWQNLVHEVWTAVIPETEAVRRIVERDGLSEAAAQSRLQSQMSGQQLVEQSHVVLSTLWEPHITQRQVEKAWALLQKRIPKTHQALD

`N sig_peptide: 1..23 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2398 peptide sequence: MAVFRSGLLVLTTPLASLAPRLA`