Identification |
HMDB Protein ID
| CDBP02439 |
Secondary Accession Numbers
| Not Available |
Name
| Isobutyryl-CoA dehydrogenase, mitochondrial |
Description
| Not Available |
Synonyms
|
- ACAD-8
- ARC42
- Activator-recruited cofactor 42 kDa component
- Acyl-CoA dehydrogenase family member 8
|
Gene Name
| ACAD8 |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in acyl-CoA dehydrogenase activity |
Specific Function
| Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.
|
GO Classification
|
Biological Process |
branched-chain amino acid catabolic process |
cellular nitrogen compound metabolic process |
lipid metabolic process |
regulation of transcription, DNA-dependent |
transcription, DNA-dependent |
valine catabolic process |
Cellular Component |
mitochondrial matrix |
Function |
oxidoreductase activity |
fad or fadh2 binding |
oxidoreductase activity, acting on the ch-ch group of donors |
acyl-coa dehydrogenase activity |
binding |
catalytic activity |
nucleoside binding |
purine nucleoside binding |
adenyl nucleotide binding |
Molecular Function |
acyl-CoA dehydrogenase activity |
flavin adenine dinucleotide binding |
Process |
metabolic process |
oxidation reduction |
|
Cellular Location
|
- Mitochondrion
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | L-valine degradation | Not Available | Not Available | Valine, Leucine and Isoleucine Degradation | | | Beta-Ketothiolase Deficiency | | Not Available | 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency | | Not Available | Propionic Acidemia | | Not Available |
|
Gene Properties |
Chromosome Location
| 11 |
Locus
| 11q25 |
SNPs
| ACAD8 |
Gene Sequence
|
>1248 bp
ATGCTGTGGAGCGGCTGCCGGCGTTTCGGGGCGCGCCTCGGCTGCCTGCCCGGCGGTCTC
CGGGTCCTCGTCCAGACCGGCCACCGGAGCTTGACCTCCTGCATCGACCCTTCCATGGGA
CTTAATGAAGAGCAGAAAGAATTTCAAAAAGTGGCCTTTGACTTTGCTGCCCGAGAGATG
GCTCCAAATATGGCAGAGTGGGACCAGAAGGAGCTGTTCCCAGTGGATGTGATGCGGAAG
GCAGCCCAGCTAGGCTTCGGAGGGGTCTACATACAAACAGATGTGGGCGGGTCTGGGCTG
TCACGTCTTGATACCTCTGTCATTTTTGAAGCCTTGGCTACAGGCTGCACCAGCACCACA
GCCTATATAAGCATCCACAACATGTGTGCCTGGATGATTGATAGCTTCGGAAATGAGGAA
CAGAGGCACAAATTTTGCCCACCGCTCTGTACCATGGAGAAGTTTGCTTCCTACTGCCTC
ACTGAACCAGGAAGTGGGAGTGATGCTGCCTCTCTTCTGACCTCCGCTAAGAAACAGGGA
GATCATTACATCCTCAATGGCTCCAAGGCCTTCATCAGTGGTGCTGGTGAGTCAGACATC
TATGTGGTCATGTGCCGAACAGGAGGACCAGGCCCCAAGGGCATCTCATGCATAGTTGTT
GAGAAGGGGACCCCTGGCCTCAGCTTTGGCAAGAAGGAGAAAAAGGTGGGGTGGAACTCC
CAGCCAACACGAGCTGTGATCTTCGAAGACTGTGCTGTCCCTGTGGCCAACAGAATTGGG
AGCGAGGGGCAGGGCTTCCTCATTGCCGTGAGAGGACTGAACGGAGGGAGGATCAATATT
GCTTCCTGCTCCCTGGGGGCTGCCCACGCCTCTGTCATCCTCACCCGAGACCACCTCAAT
GTCCGGAAGCAGTTTGGAGAGCCTCTGGCCAGTAACCAGTACTTGCAATTCACACTGGCT
GATATGGCAACAAGGCTGGTGGCCGCGCGGCTGATGGTCCGCAATGCAGCAGTGGCTCTG
CAGGAGGAGAGGAAGGATGCAGTGGCCTTGTGCTCCATGGCCAAGCTCTTTGCTACAGAT
GAATGCTTTGCCATCTGCAACCAGGCCTTGCAGATGCACGGGGGCTACGGCTACCTGAAG
GATTACGCTGTTCAGCAGTACGTGCGGGACTCCAGGGTCCACCAGATTCTAGAAGGTAGC
AATGAAGTGATGAGGATACTGATCTCTAGAAGCCTGCTTCAGGAGTAG
|
Protein Properties |
Number of Residues
| 415 |
Molecular Weight
| 45069.39 |
Theoretical pI
| 7.849 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Isobutyryl-CoA dehydrogenase, mitochondrial
MLWSGCRRFGARLGCLPGGLRVLVQTGHRSLTSCIDPSMGLNEEQKEFQKVAFDFAAREM
APNMAEWDQKELFPVDVMRKAAQLGFGGVYIQTDVGGSGLSRLDTSVIFEALATGCTSTT
AYISIHNMCAWMIDSFGNEEQRHKFCPPLCTMEKFASYCLTEPGSGSDAASLLTSAKKQG
DHYILNGSKAFISGAGESDIYVVMCRTGGPGPKGISCIVVEKGTPGLSFGKKEKKVGWNS
QPTRAVIFEDCAVPVANRIGSEGQGFLIAVRGLNGGRINIASCSLGAAHASVILTRDHLN
VRKQFGEPLASNQYLQFTLADMATRLVAARLMVRNAAVALQEERKDAVALCSMAKLFATD
ECFAICNQALQMHGGYGYLKDYAVQQYVRDSRVHQILEGSNEVMRILISRSLLQE
|
External Links |
GenBank ID Protein
| Not Available |
UniProtKB/Swiss-Prot ID
| Q9UKU7 |
UniProtKB/Swiss-Prot Entry Name
| ACAD8_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| AF126245 |
GeneCard ID
| ACAD8 |
GenAtlas ID
| ACAD8 |
HGNC ID
| HGNC:87 |
References |
General References
| Not Available |