Gene/Proteome Database (LMPD)
LMPD ID
LMP000751
Gene ID
Species
Homo sapiens (Human)
Gene Name
glutaryl-CoA dehydrogenase
Gene Symbol
Synonyms
ACAD5; GCD
Alternate Names
glutaryl-CoA dehydrogenase, mitochondrial; glutaryl-Coenzyme A dehydrogenase
Chromosome
19
Map Location
19p13.2
EC Number
1.3.8.6
Summary
The 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]
Orthologs
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 |
| MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK | |
| 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 |
| MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK | |
| 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: RPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK 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 | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005743 | IEA:Ensembl | C | mitochondrial inner membrane |
| GO:0005759 | TAS:Reactome | C | mitochondrial matrix |
| GO:0005739 | IDA:HPA | C | mitochondrion |
| GO:0000062 | IEA:Ensembl | F | fatty-acyl-CoA binding |
| GO:0050660 | IEA:Ensembl | F | flavin adenine dinucleotide binding |
| GO:0004361 | EXP:Reactome | F | glutaryl-CoA dehydrogenase activity |
| GO:0034641 | TAS:Reactome | P | cellular nitrogen compound metabolic process |
| GO:0019395 | IEA:Ensembl | P | fatty acid oxidation |
| GO:0046949 | IEA:Ensembl | P | fatty-acyl-CoA biosynthetic process |
| GO:0006554 | TAS:Reactome | P | lysine catabolic process |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0006568 | IEA:UniProtKB-UniPathway | P | tryptophan metabolic process |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY-5177 | glutaryl-CoA degradation |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_1298 | Lysine catabolism |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR006089 | Acyl-CoA dehydrogenase, conserved site |
| IPR009075 | Acyl-CoA dehydrogenase/oxidase C-terminal |
| IPR013786 | Acyl-CoA dehydrogenase/oxidase, N-terminal |
| IPR009100 | Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain |
| IPR006091 | Acyl-CoA oxidase/dehydrogenase, central domain |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=2; Name=Long; IsoId=Q92947-1; Sequence=Displayed; Name=Short; IsoId=Q92947-2; Sequence=VSP_000145; |
| Biophysicochemical Properties | Kinetic parameters: KM=4.7 uM for glutaryl-CoA (at pH 6.5) ; KM=5.5 uM for glutaryl-CoA (at pH 7.5) ; KM=8.1 uM for glutaryl-CoA (at pH 7.6) ; KM=34.0 uM for glutaryl-CoA (at pH 8.5) ; Note=Release of crotonyl-CoA product from the enzyme is the rate-determining step in its steady-state turnover.; |
| Catalytic Activity | Glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO(2) + reduced electron-transfer flavoprotein. |
| Cofactor | Name=FAD; Xref=ChEBI |
| Disease | Glutaric aciduria 1 (GA1) [MIM |
| Enzyme Regulation | Strongly inhibited by MCPA-CoA, a metabolite of hypoglycin which is present in unripened fruit of the ackee tree. |
| Function | 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. Isoform Short is inactive. {ECO |
| Interaction | P62993:GRB2; NbExp=1; IntAct=EBI-1236978, EBI-401755; P29474:NOS3; NbExp=1; IntAct=EBI-1236978, EBI-1391623; P49768:PSEN1; NbExp=1; IntAct=EBI-1236978, EBI-297277; |
| Pathway | Amino-acid metabolism; lysine degradation. |
| Pathway | Amino-acid metabolism; tryptophan metabolism. |
| Similarity | Belongs to the acyl-CoA dehydrogenase family. |
| Subcellular Location | Mitochondrion matrix. |
| Subunit | Homotetramer. {ECO |
| Tissue Specificity | Isoform 1 and isoform 2 are expressed in fibroblasts and liver. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000751 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 4503943 | RefSeq | NP_000150 | 438 | glutaryl-CoA dehydrogenase, mitochondrial isoform a precursor |
| 4503943 | RefSeq | NP_039663 | 438 | glutaryl-CoA dehydrogenase, mitochondrial isoform b precursor |
Identical Sequences to LMP000751 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:4503943 | GenBank | AEQ84582.1 | 438 | Sequence 76 from patent US 8039587 |
| GI:4503943 | GenBank | AEQ84582.1 | 438 | Sequence 76 from patent US 8039587 |
| GI:4503943 | GenBank | AEU53445.1 | 438 | Sequence 76 from patent US 8062891 |
| GI:4503943 | GenBank | AEU53445.1 | 438 | Sequence 76 from patent US 8062891 |
| GI:4503943 | GenBank | AGO72398.1 | 438 | Sequence 76 from patent US 8470972 |
| GI:4503943 | GenBank | AGO72398.1 | 438 | Sequence 76 from patent US 8470972 |
| GI:4503943 | GenBank | AGU32389.1 | 438 | Sequence 76 from patent US 8507277 |
| GI:4503943 | GenBank | AGU32389.1 | 438 | Sequence 76 from patent US 8507277 |
| GI:4503943 | GenBank | AGX48293.1 | 438 | Sequence 76 from patent US 8541550 |
| GI:4503943 | GenBank | AGX48293.1 | 438 | Sequence 76 from patent US 8541550 |
| GI:4503943 | GenBank | AIC48816.1 | 438 | GCDH, partial [synthetic construct] |
| GI:4503943 | GenBank | AIC48816.1 | 438 | GCDH, partial [synthetic construct] |
Related Sequences to LMP000751 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:4503943 | GenBank | AAP36673.1 | 439 | Homo sapiens glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | AAP36673.1 | 439 | Homo sapiens glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | AAX43755.1 | 439 | glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | AAX43755.1 | 439 | glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | AAX43756.1 | 439 | glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | AAX43756.1 | 439 | glutaryl-Coenzyme A dehydrogenase, partial [synthetic construct] |
| GI:4503943 | GenBank | ACM82874.1 | 440 | Sequence 8372 from patent US 6812339 |
| GI:4503943 | GenBank | ACM82874.1 | 440 | Sequence 8372 from patent US 6812339 |
| GI:4503943 | GenBank | ACM82875.1 | 440 | Sequence 8373 from patent US 6812339 |
| GI:4503943 | GenBank | ACM82875.1 | 440 | Sequence 8373 from patent US 6812339 |
| GI:4503943 | GenBank | JAA21165.1 | 438 | glutaryl-CoA dehydrogenase [Pan troglodytes] |
| GI:4503943 | GenBank | JAA21165.1 | 438 | glutaryl-CoA dehydrogenase [Pan troglodytes] |