Acadl
entrez
11363
canonical
uniprot
P51174
canonical
ensembl
ENSMUSG00000026003
resolver
ensp
ENSMUSP00000027153
resolver
enst
ENSMUST00000027153
resolver
genesymbol
Acadl
resolver
uniprot
A0A0R4J083
resolver
uniprot
B2KGC6
resolver
uniprot
O35302
resolver
uniprot
Q8QZR6
resolver
uniprot
Q9CU29
resolver
uniprot
Q9DB83
resolver
uniprot_entry
A0A0R4J083_MOUSE
resolver
uniprot_entry
ACADL_MOUSE
resolver
uniprot_sec
B2KGC6
resolver
uniprot_sec
O35302
resolver
uniprot_sec
Q8QZR6
resolver
uniprot_sec
Q9CU29
resolver
uniprot_sec
Q9DB83
resolver
amino_acid_sequence
MAARLLLRSLRVLKARSAPRPPPSARCSHSGAEARLETPSAKKLTDVGIRRIFSSEHDIFRESVRKFFQEEVIPHHTEWEKAGEVSREVWEKAGKQGLLGINIAEKHGGIGGDLLSTAVTWEEQAYSNCTGPGFSLHSDIVMPYIANYGTKEQIEKFIPQMTAGKCIGAIAMTEPGAGSDLQGVRTNAKRSGSDWILNGSKVFITNGWLSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKGRKLHKMGMKAQDTAELFFEDVRLPANALLGEENKGFYYLMQELPQERLLIAELAISACEFMFEETRNYVKQRKAFGKTVAHIQTVQHKLAELKTHICVTRAFVDSCLQLHETKRLDSGSASMAKYWASELQNSVAYECVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIARQIVSDS
uniprot
proteins
ec_number
1.3.8.7
uniprot
proteins
ec_number
1.3.8.8
uniprot
proteins
function
FUNCTION: Catalyzes the first of the four reactions of the mitochondrial fatty acid beta-oxidation (FAO) pathway, which consists in the proR-proR stereospecific alpha, beta-dehydrogenation of fatty acyl-CoA thioesters using the electron transfer flavoprotein (ETF) as their physiologic electron acceptor, resulting in the formation of trans-2-enoyl-CoA ((2E)-enoyl-CoA). The mitochondrial FAO pathway is the major energy-producing process in tissues and is performed through cycles of four consecutive reactions. Each FAO cycle shortens the fatty acyl-CoA by two carbons, yielding one acetyl-CoA (for the citric acid cycle), one FADH(2), and one NADH (which donate electrons to the respiratory chain for ATP production) (By similarity). Among the different mitochondrial acyl-CoA dehydrogenases, long-chain specific acyl-CoA dehydrogenase activity overlaps with that of ACADV and ACAD9, acting on saturated and unsaturated acyl-CoAs with 6 to 24 carbons with a preference for 8 to 18 carbons long primary chains (PubMed:9861014). Can use (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate as substrate in vitro (which is not primarily used for energy but mainly beta-oxidized in the peroxisomes). In addition, based on its established catalytic mechanism, and combined genetic interaction or mutant phenotype evidence, it is predicted to act also on substrates that have not been tested experimentally but are metabolized by mitochondrial FAO, including long-chain unsaturated fatty acids such as linoleate (9Z,12Z-octadecadienoate), linolenate (9Z,12Z,15Z-octadecatrienoate), and others. Plays a primary role in FAO in tissues where it is the main long-chain ACAD expressed. Probably responsible for beta-oxidation of bulky substrates including branched chain fatty acyl-CoAs and sterol derivatives thanks to its enlarged substrate-binding cavity (By similarity). {ECO:0000250|UniProtKB:P28330, ECO:0000269|PubMed:9861014}.
uniprot
proteins
mass_dalton
47908
uniprot
proteins
mutagenesis
MUTAGEN 42; /note="K->R: Reduces activity by 90% when associated with R-318 and R-322."; /evidence="ECO:0000269|PubMed:24121500"; MUTAGEN 318; /note="K->R: Reduces activity by 37%; reduces activity by 80% when associated with R-322."; /evidence="ECO:0000269|PubMed:24121500"; MUTAGEN 322; /note="K->R: Reduces activity by 23%; reduces activity by 80% when associated with R-318."; /evidence="ECO:0000269|PubMed:24121500
uniprot
proteins
pathway_participation
PATHWAY: Lipid metabolism; mitochondrial fatty acid beta-oxidation. {ECO:0000269|PubMed:9861014}.
uniprot
proteins
post_translational_modification
PTM: Acetylation at Lys-318 and Lys-322 in proximity of the cofactor-binding sites strongly reduces catalytic activity. These sites are deacetylated by SIRT3. {ECO:0000269|PubMed:24121500}.
uniprot
proteins
protein_family
Acyl-CoA dehydrogenase family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
19468303
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
23576753
uniprot
proteins
pubmed
23806337
uniprot
proteins
pubmed
24121500
uniprot
proteins
pubmed
8530022
uniprot
proteins
pubmed
9545492
uniprot
proteins
pubmed
9861014
uniprot
proteins
sequence_length
430
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000250|UniProtKB:P15650}.
uniprot
proteins