MGP Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
MGP001173

Record overview

MGPD IDMGP001173
Gene ID2639
SpeciesHomo sapiens (Human)
Gene Nameglutaryl-CoA dehydrogenase
Gene Symbol GCDH
SynonymsGCD; ACAD5;
Alternate namesglutaryl-CoA dehydrogenase, mitochondrial; glutaryl-Coenzyme A dehydrogenase;
Chromosome19
Map Location19p13.2
EC Number1.3.8.6
SummaryThe protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]
OrthologsView orthologs and multiple alignments for GCDH

Proteins

glutaryl-CoA dehydrogenase, mitochondrial isoform a precursor
Refseq ID:NP_000150
Protein GI:4503943
UniProt ID:Q92947
mRNA ID:NM_000159
Length:438
RefSeq Status:
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEM
GELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSS
NKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWG
VLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHV
IRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK
 
transit_peptide: 1..44
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (Potential); propagated from UniProtKB/Swiss-Prot (Q92947.1)
calculated_mol_wt: 4678
peptide sequence: 
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSS

mat_peptide: 45..438
product: Glutaryl-CoA dehydrogenase, mitochondrial
experiment: experimental evidence, no additional details recorded
note: propagated from UniProtKB/Swiss-Prot (Q92947.1)
calculated_mol_wt: 43467
peptide sequence: 
RPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQ
SSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRG
LSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEIT
LGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK
 
glutaryl-CoA dehydrogenase, mitochondrial isoform b precursor
Refseq ID:NP_039663
Protein GI:7669494
UniProt ID:Q92947
mRNA ID:NM_013976
Length:428
RefSeq Status:
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEM
GELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSS
NKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWG
VLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHV
IRHAMNLEAVNTYEVVQMCSLKRRWNSL
 
transit_peptide: 1..44
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (Potential); propagated from UniProtKB/Swiss-Prot (Q92947.1)
calculated_mol_wt: 4678
peptide sequence: 
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSS

mat_peptide: 45..438
product: Glutaryl-CoA dehydrogenase, mitochondrial
experiment: experimental evidence, no additional details recorded
note: propagated from UniProtKB/Swiss-Prot (Q92947.1)
calculated_mol_wt: 43467
peptide sequence: 
RPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQ
SSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRG
LSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEIT
LGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK

transit_peptide: 1..44
inference: non-experimental evidence, no additional details recorded
note: Mitochondrion (Potential); propagated from UniProtKB/Swiss-Prot (Q92947.1)
calculated_mol_wt: 4678
peptide sequence: 
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSS
 
  logo