Gene/Proteome Database (LMPD)
LMPD ID
LMP000014
Gene ID
Species
Homo sapiens (Human)
Gene Name
acyl-CoA dehydrogenase family, member 9
Gene Symbol
Synonyms
NPD002
Alternate Names
acyl-CoA dehydrogenase family member 9, mitochondrial; very-long-chain acyl-CoA dehydrogenase VLCAD; acyl-Coenzyme A dehydrogenase family, member 9
Chromosome
3
Map Location
3q21.3
EC Number
1.3.99.-
Summary
This gene encodes a member of the acyl-CoA dehydrogenase family. Members of this family of proteins localize to the mitochondria and catalyze the rate-limiting step in the beta-oxidation of fatty acyl-CoA. The encoded protein is specifically active toward palmitoyl-CoA and long-chain unsaturated substrates. Mutations in this gene cause acyl-CoA dehydrogenase family member type 9 deficiency. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Mar 2010]
Orthologs
Proteins
| acyl-CoA dehydrogenase family member 9, mitochondrial | |
|---|---|
| Refseq ID | NP_054768 |
| Protein GI | 21361497 |
| UniProt ID | Q9H845 |
| mRNA ID | NM_014049 |
| Length | 621 |
| RefSeq Status | REVIEWED |
| MSGCGLFLRTTAAARACRGLVVSTANRRLLRTSPPVRAFAKELFLGKIKKKEVFPFPEVSQDELNEINQFLGPVEKFFTEEVDSRKIDQEGKIPDETLEKLKSLGLFGLQVPEEYGGLGFSNTMYSRLGEIISMDGSITVTLAAHQAIGLKGIILAGTEEQKAKYLPKLASGEHIAAFCLTEPASGSDAASIRSRATLSEDKKHYILNGSKVWITNGGLANIFTVFAKTEVVDSDGSVKDKITAFIVERDFGGVTNGKPEDKLGIRGSNTCEVHFENTKIPVENILGEVGDGFKVAMNILNSGRFSMGSVVAGLLKRLIEMTAEYACTRKQFNKRLSEFGLIQEKFALMAQKAYVMESMTYLTAGMLDQPGFPDCSIEAAMVKVFSSEAAWQCVSEALQILGGLGYTRDYPYERILRDTRILLIFEGTNEILRMYIALTGLQHAGRILTTRIHELKQAKVSTVMDTVGRRLRDSLGRTVDLGLTGNHGVVHPSLADSANKFEENTYCFGRTVETLLLRFGKTIMEEQLVLKRVANILINLYGMTAVLSRASRSIRIGLRNHDHEVLLANTFCVEAYLQNLFSLSQLDKYAPENLDEQIKKVSQQILEKRAYICAHPLDRTC | |
Gene Information
Entrez Gene ID
Gene Name
acyl-CoA dehydrogenase family, member 9
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0030425 | IDA:UniProtKB | C | dendrite |
| GO:0005739 | IDA:LIFEdb | C | mitochondrion |
| GO:0005634 | IDA:UniProtKB | C | nucleus |
| GO:0003995 | IEA:InterPro | F | acyl-CoA dehydrogenase activity |
| GO:0050660 | IEA:InterPro | F | flavin adenine dinucleotide binding |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR006089 | Acyl-CoA dehydrogenase, conserved site |
| IPR009075 | Acyl-CoA dehydrogenase/oxidase C-terminal |
| IPR013786 | Acyl-CoA dehydrogenase/oxidase, N-terminal |
| IPR009100 | Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain |
| IPR006091 | Acyl-CoA oxidase/dehydrogenase, central domain |
UniProt Annotations
Entry Information
Gene Name
acyl-CoA dehydrogenase family, member 9
Protein Entry
ACAD9_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Cofactor | Name=FAD; Xref=ChEBI |
| Disease | Acyl-CoA dehydrogenase family, member 9, deficiency (ACAD9 deficiency) [MIM |
| Function | Has a dehydrogenase activity on palmitoyl-CoA (C16:0) and stearoyl-CoA (C18:0). It is three times more active on palmitoyl-CoA than on stearoyl-CoA. Has little activity on octanoyl-CoA (C8:0), butyryl-CoA (C4:0) or isovaleryl-CoA (5:0). |
| Similarity | Belongs to the acyl-CoA dehydrogenase family. |
| Subcellular Location | Mitochondrion . |
| Subunit | Part of the mitochondrial complex I assembly (MCIA) complex. The complex comprises at least TMEM126B, NDUFAF1, ECSIT, and ACAD9 (By similarity). |
| Tissue Specificity | Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000014 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 21361497 | RefSeq | NP_054768 | 621 | acyl-CoA dehydrogenase family member 9, mitochondrial |
Identical Sequences to LMP000014 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:21361497 | GenBank | JAA04203.1 | 621 | acyl-CoA dehydrogenase family, member 9 [Pan troglodytes] |
| GI:21361497 | GenBank | JAA18217.1 | 621 | acyl-CoA dehydrogenase family, member 9 [Pan troglodytes] |
| GI:21361497 | GenBank | JAA23477.1 | 621 | acyl-CoA dehydrogenase family, member 9 [Pan troglodytes] |
| GI:21361497 | GenBank | JAA41804.1 | 621 | acyl-CoA dehydrogenase family, member 9 [Pan troglodytes] |
| GI:21361497 | GenBank | AIC51242.1 | 621 | ACAD9, partial [synthetic construct] |
| GI:21361497 | RefSeq | XP_003829456.1 | 621 | PREDICTED: acyl-CoA dehydrogenase family member 9, mitochondrial isoform X1 [Pan paniscus] |
Related Sequences to LMP000014 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:21361497 | GenBank | AAL56011.1 | 621 | very-long-chain acyl-CoA dehydrogenase VLCAD [Homo sapiens] |
| GI:21361497 | GenBank | AIC56261.1 | 621 | ACAD9, partial [synthetic construct] |
| GI:21361497 | RefSeq | XP_002813203.1 | 621 | PREDICTED: acyl-CoA dehydrogenase family member 9, mitochondrial isoform X1 [Pongo abelii] |
| GI:21361497 | RefSeq | XP_003279947.1 | 621 | PREDICTED: acyl-CoA dehydrogenase family member 9, mitochondrial isoform 1 [Nomascus leucogenys] |
| GI:21361497 | RefSeq | XP_004036310.1 | 621 | PREDICTED: acyl-CoA dehydrogenase family member 9, mitochondrial isoform 1 [Gorilla gorilla gorilla] |
| GI:21361497 | RefSeq | XP_010352967.1 | 621 | PREDICTED: acyl-CoA dehydrogenase family member 9, mitochondrial [Rhinopithecus roxellana] |