| 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 inner membrane |
| mitochondrial nucleoid |
| 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 |