Identification |
HMDB Protein ID
| CDBP00197 |
Secondary Accession Numbers
| Not Available |
Name
| Glutaryl-CoA dehydrogenase, mitochondrial |
Description
| Not Available |
Synonyms
|
- GCD
|
Gene Name
| GCDH |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in acyl-CoA dehydrogenase activity |
Specific 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.
|
GO Classification
|
Biological Process |
cellular nitrogen compound metabolic process |
lysine catabolic process |
fatty acid oxidation |
fatty-acyl-CoA biosynthetic process |
tryptophan metabolic process |
Cellular Component |
mitochondrial matrix |
mitochondrial inner membrane |
Function |
nucleoside binding |
purine nucleoside binding |
adenyl nucleotide binding |
oxidoreductase activity |
fad or fadh2 binding |
oxidoreductase activity, acting on the ch-ch group of donors |
acyl-coa dehydrogenase activity |
binding |
catalytic activity |
Molecular Function |
glutaryl-CoA dehydrogenase activity |
flavin adenine dinucleotide binding |
fatty-acyl-CoA binding |
Process |
metabolic process |
oxidation reduction |
|
Cellular Location
|
- Mitochondrion matrix
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | Fatty acid Metabolism | | | Ethylmalonic Encephalopathy | | Not Available | Glutaric Aciduria Type I | | Not Available | Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency) | | Not Available | Carnitine palmitoyl transferase deficiency (I) | | Not Available |
|
Gene Properties |
Chromosome Location
| 19 |
Locus
| 19p13.2 |
SNPs
| GCDH |
Gene Sequence
|
>1317 bp
ATGGCCCTGAGAGGCGTCTCCGTGCGGCTGCTGAGCCGCGGACCCGGCCTGCACGTCCTT
CGCACGTGGGTCTCGTCGGCGGCGCAGACCGAGAAAGGCGGGAGAACACAGAGCCAACTG
GCTAAGTCCTCGCGTCCCGAGTTTGACTGGCAGGACCCGCTGGTGCTGGAGGAGCAGCTG
ACCACAGATGAGATCCTCATCAGGGACACCTTCCGCACCTACTGCCAGGAGAGACTCATG
CCTCGCATCCTGTTGGCCAATCGCAACGAAGTTTTTCATCGGGAGATCATTTCGGAGATG
GGGGAGTTGGGTGTGCTGGGCCCCACCATCAAAGGATATGGCTGTGCTGGGGTTTCGTCT
GTGGCCTATGGGCTCCTGGCCCGAGAGCTGGAGCGGGTGGACAGTGGCTACAGGTCGGCG
ATGAGTGTCCAGTCCTCCCTCGTCATGCACCCTATCTATGCCTATGGCAGCGAGGAACAG
CGGCAGAAGTACCTGCCCCAGCTGGCCAAGGGGGAGCTCCTGGGCTGCTTCGGGCTCACA
GAGCCCAACAGCGGAAGTGACCCCAGCAGCATGGAGACCAGAGCCCACTACAACTCATCC
AACAAGAGCTACACCCTCAATGGGACCAAGACCTGGATCACGAACTCGCCTATGGCCGAT
CTGTTTGTAGTGTGGGCTCGGTGTGAAGATGGCTGCATTCGGGGCTTCCTGCTGGAGAAG
GGGATGCGGGGTCTCTCGGCCCCCAGGATCCAGGGCAAGTTCTCGCTGCGGGCCTCAGCC
ACAGGCATGATCATCATGGACGGTGTGGAGGTGCCAGAGGAGAATGTGCTCCCTGGTGCA
TCCAGCCTGGGGGGTCCCTTCGGCTGCCTGAACAACGCCCGGTACGGCATCGCGTGGGGC
GTGCTTGGAGCTTCGGAGTTCTGCTTGCACACAGCCCGGCAGTACGCCCTCGACAGGATG
CAGTTTGGTGTCCCACTGGCCAGGAACCAGCTGATTCAGAAGAAGCTGGCAGACATGCTC
ACTGAGATTACCCTGGGCCTTCACGCCTGCCTGCAGCTCGGCCGCTTGAAGGACCAGGAC
AAGGCTGCCCCCGAGATGGTTTCTCTGCTGAAGAGGAATAACTGTGGGAAAGCCCTGGAC
ATCGCCCGCCAGGCCCGAGACATGCTGGGGGGGAATGGGATTTCTGACGAGTATCACGTG
ATCCGGCACGCCATGAACCTGGAGGCCGTGAACACCTACGAAGGTACACATGACATTCAC
GCCCTGATCCTTGGGAGAGCTATCACGGGAATCCAGGCGTTCACGGCCAGCAAGTGA
|
Protein Properties |
Number of Residues
| 438 |
Molecular Weight
| 48126.715 |
Theoretical pI
| 8.062 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Glutaryl-CoA dehydrogenase, mitochondrial
MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQL
TTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSS
VAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLT
EPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEK
GMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWG
VLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQD
KAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIH
ALILGRAITGIQAFTASK
|
External Links |
GenBank ID Protein
| Not Available |
UniProtKB/Swiss-Prot ID
| Q92947 |
UniProtKB/Swiss-Prot Entry Name
| GCDH_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| U69141 |
GeneCard ID
| GCDH |
GenAtlas ID
| GCDH |
HGNC ID
| HGNC:4189 |
References |
General References
| Not Available |