Identification |
HMDB Protein ID
| CDBP03473 |
Secondary Accession Numbers
| Not Available |
Name
| Very long-chain specific acyl-CoA dehydrogenase, mitochondrial |
Description
| Not Available |
Synonyms
|
- VLCAD
|
Gene Name
| ACADVL |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in acyl-CoA dehydrogenase activity |
Specific Function
| Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
|
GO Classification
|
Biological Process |
fatty acid beta-oxidation using acyl-CoA dehydrogenase |
negative regulation of fatty acid biosynthetic process |
negative regulation of fatty acid oxidation |
regulation of cholesterol metabolic process |
temperature homeostasis |
activation of signaling protein activity involved in unfolded protein response |
energy derivation by oxidation of organic compounds |
very long-chain fatty acid catabolic process |
Cellular Component |
mitochondrial nucleoid |
mitochondrial inner membrane |
Function |
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 |
oxidoreductase activity |
fad or fadh2 binding |
Molecular Function |
flavin adenine dinucleotide binding |
fatty-acyl-CoA binding |
long-chain-acyl-CoA dehydrogenase activity |
very-long-chain-acyl-CoA dehydrogenase activity |
Process |
metabolic process |
oxidation reduction |
|
Cellular Location
|
- Mitochondrion inner membrane
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | Fatty acid Metabolism | | | Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids | | Not Available | mitochondrial fatty acid beta-oxidation | Not Available | Not Available | Ethylmalonic Encephalopathy | | Not Available | Glutaric Aciduria Type I | | Not Available |
|
Gene Properties |
Chromosome Location
| 17 |
Locus
| 17p13.1 |
SNPs
| ACADVL |
Gene Sequence
|
>1968 bp
ATGCAGGCGGCTCGGATGGCCGCGAGCTTGGGGCGGCAGCTGCTGAGGCTCGGGGGCGGA
AGCTCGCGGCTCACGGCGCTCCTGGGGCAGCCCCGGCCCGGCCCTGCCCGGCGGCCCTAT
GCCGGGGGTGCCGCTCAGCTGGCTCTGGACAAGTCAGATTCCCACCCCTCTGACGCTCTG
ACCAGGAAAAAACCGGCCAAGGCGGAATCTAAGTCCTTTGCTGTGGGAATGTTCAAAGGC
CAGCTCACCACAGATCAGGTGTTCCCATACCCGTCCGTGCTCAACGAAGAGCAGACACAG
TTTCTTAAAGAGCTGGTGGAGCCTGTGTCCCGTTTCTTCGAGGAAGTGAACGATCCCGCC
AAGAATGACGCTCTGGAGATGGTGGAGGAGACCACTTGGCAGGGCCTCAAGGAGCTGGGG
GCCTTTGGTCTGCAAGTGCCCAGTGAGCTGGGTGGTGTGGGCCTTTGCAACACCCAGTAC
GCCCGTTTGGTGGAGATCGTGGGCATGCATGACCTTGGCGTGGGCATTACCCTGGGGGCC
CATCAGAGCATCGGTTTCAAAGGCATCCTGCTCTTTGGCACAAAGGCCCAGAAAGAAAAA
TACCTCCCCAAGCTGGCATCTGGGGAGACTGTGGCCGCTTTCTGTCTAACCGAGCCCTCA
AGCGGGTCAGATGCAGCCTCCATCCGAACCTCTGCTGTGCCCAGCCCCTGTGGAAAATAC
TATACCCTCAATGGAAGCAAGCTTTGGATCAGTAATGGGGGCCTAGCAGACATCTTCACG
GTCTTTGCCAAGACACCAGTTACAGATCCAGCCACAGGAGCCGTGAAGGAGAAGATCACA
GCTTTTGTGGTGGAGAGGGGCTTCGGGGGCATTACCCATGGGCCCCCTGAGAAGAAGATG
GGCATCAAGGCTTCAAACACAGCAGAGGTGTTCTTTGATGGAGTACGGGTGCCATCGGAG
AACGTGCTGGGTGAGGTTGGGAGTGGCTTCAAGGTTGCCATGCACATCCTCAACAATGGA
AGGTTTGGCATGGCTGCGGCCCTGGCAGGTACCATGAGAGGCATCATTGCTAAGGCGGTA
GATCATGCCACTAATCGTACCCAGTTTGGGGAGAAAATTCACAACTTTGGGCTGATCCAG
GAGAAGCTGGCACGGATGGTTATGCTGCAGTATGTAACTGAGTCCATGGCTTACATGGTG
AGTGCTAACATGGACCAGGGAGCCACGGACTTCCAGATAGAGGCCGCCATCAGCAAAATC
TTTGGCTCGGAGGCAGCCTGGAAGGTGACAGATGAATGCATCCAAATCATGGGGGGTATG
GGCTTCATGAAGGAACCTGGAGTAGAGCGTGTGCTCCGAGATCTTCGCATCTTCCGGATC
TTTGAGGGGACAAATGACATTCTTCGGCTGTTTGTGGCTCTGCAGGGCTGTATGGACAAA
GGAAAGGAGCTCTCTGGGCTTGGCAGTGCTCTAAAGAATCCCTTTGGGAATGCTGGCCTC
CTGCTAGGAGAGGCAGGCAAACAGCTGAGGCGGCGGGCAGGGCTGGGCAGCGGCCTGAGT
CTCAGCGGACTTGTCCACCCGGAGTTGAGTCGGAGTGGCGAGCTGGCAGTACGGGCTCTG
GAGCAGTTTGCCACTGTGGTGGAGGCCAAGCTGATAAAACACAAGAAGGGGATTGTCAAT
GAACAGTTTCTGCTGCAGCGGCTGGCAGACGGGGCCATCGACCTCTATGCCATGGTGGTG
GTTCTCTCGAGGGCCTCAAGATCCCTGAGTGAGGGCCACCCCACGGCCCAGCATGAGAAA
ATGCTCTGTGACACCTGGTGTATCGAGGCTGCAGCTCGGATCCGAGAGGGCATGGCCGCC
CTGCAGTCTGACCCCTGGCAGCAAGAGCTCTACCGCAACTTCAAAAGCATCTCCAAGGCC
TTGGTGGAGCGGGGTGGTGTGGTCACCAGCAACCCACTTGGCTTCTGA
|
Protein Properties |
Number of Residues
| 655 |
Molecular Weight
| 70389.58 |
Theoretical pI
| 8.755 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
MQAARMAASLGRQLLRLGGGSSRLTALLGQPRPGPARRPYAGGAAQLALDKSDSHPSDAL
TRKKPAKAESKSFAVGMFKGQLTTDQVFPYPSVLNEEQTQFLKELVEPVSRFFEEVNDPA
KNDALEMVEETTWQGLKELGAFGLQVPSELGGVGLCNTQYARLVEIVGMHDLGVGITLGA
HQSIGFKGILLFGTKAQKEKYLPKLASGETVAAFCLTEPSSGSDAASIRTSAVPSPCGKY
YTLNGSKLWISNGGLADIFTVFAKTPVTDPATGAVKEKITAFVVERGFGGITHGPPEKKM
GIKASNTAEVFFDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAV
DHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKI
FGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDK
GKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRAL
EQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEK
MLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF
|
External Links |
GenBank ID Protein
| 12653261 |
UniProtKB/Swiss-Prot ID
| P49748 |
UniProtKB/Swiss-Prot Entry Name
| ACADV_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| BC000399 |
GeneCard ID
| ACADVL |
GenAtlas ID
| ACADVL |
HGNC ID
| HGNC:92 |
References |
General References
| Not Available |