| 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 |
| binding |
| catalytic activity |
| 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 |
| 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 |