Overview
| Uniprot ID | P79274 |
|---|
| Protein Name | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
|---|
| Gene Name | ACADL |
|---|
| Organism | Sus scrofa |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 43 |
LESPSAKKLTDIGIR |
| 66 |
IFRESVRKFFQEEVI |
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). 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. 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
Protein Sequence
10
MATRLLRGSL
20
RLWGGLCAPR
30
LPTASRCSHS
40
GGEERLESPS
50
AKKLTDIGIR
60
RIFSSEHDIF
70
RESVRKFFQE
80
EVIPHHAEWE
90
KAGEVSRELW
100
EKAGKQGLLG
110
INIAERHGGI
120
GGDLYSAAIV
130
WEEQAYSNCT
140
GPGFSLHSDI
150
VMPYIANYGS
160
EEQIKHFIPQ
170
MTAGKCIGAI
180
AMTEPGAGSD
190
LQGVRTNAKK
200
DGSDWILNGS
210
KVFITNGWLS
220
DVVIVVAVTN
230
REARSPAHGI
240
SLFLVENGMK
250
GFVKGRKLHK
260
IGLKAQDTAE
270
LFFEDVRLPA
280
SALLGEENKG
290
FYYLMQELPQ
300
ERLLIAELAV
310
SASEFMFEET
320
RNYVKQRKAF
330
GKTVAHIQTV
340
QHKLAELKTH
350
ICVTRAFVDS
360
CLQLHETKRL
370
ESAAASMAKY
380
WASELQNSVA
390
YDCVQLHGGW
400
GYMWEYPIAR
410
AYVDARVQPI
420
YGGTNEIMKE
430
LIAREIVHDK
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0005737 |
cytoplasm |
| Cellular Component |
GO:0005759 |
mitochondrial matrix |
| Cellular Component |
GO:0005739 |
mitochondrion |
| Molecular Function |
GO:0050660 |
flavin adenine dinucleotide binding |
| Molecular Function |
GO:0004466 |
long-chain fatty acyl-CoA dehydrogenase activity |
| Molecular Function |
GO:0070991 |
medium-chain fatty acyl-CoA dehydrogenase activity |
| Biological Process |
GO:0019254 |
carnitine metabolic process, CoA-linked |
| Biological Process |
GO:0033539 |
fatty acid beta-oxidation using acyl-CoA dehydrogenase |
| Biological Process |
GO:0042758 |
long-chain fatty acid catabolic process |
Reference
[1] Fan S, Zhou R, Wen H, Ye H, Ma S et al.. Global profiling of protein lactylome in porcine granulosa cells.. J Ovarian Res 18(1):177. 2025 Aug 8. PMID: 40781717.