LMPD Database

LMP000751

Record overview

LMPD IDLMP000751
Gene ID2639
SpeciesHomo sapiens (Human)
Gene Nameglutaryl-CoA dehydrogenase
Gene SymbolGCDH
SynonymsACAD5; GCD
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:REVIEWED
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEM
GELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSS
NKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWG
VLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHV
IRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK
 
glutaryl-CoA dehydrogenase, mitochondrial isoform b precursor
Refseq ID:NP_039663
Protein GI:4503943
UniProt ID:Q92947
mRNA ID:NM_013976
Length:438
RefSeq Status:REVIEWED
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 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