Search Results

Overview

Uniprot ID	P50544
Protein Name	Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Name	Acadvl
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
122	EEVNDPAKNDALEKV
128	AKNDALEKVEDDTLQ
196	GILLYGTKAQREKYL
240	AIPSPCGKYYTLNGS
269	VFAKTPIKDAATGAV
277	DAATGAVKEKITAFV
279	ATGAVKEKITAFVVE
299	VTHGLPEKKMGIKAS
359	TMKSLIAKAVDHATN
373	NRTQFGDKIHNFGVI
383	NFGVIQEKLARMAIL
483	QGCMDKGKELTGLGN
52	ATQAVLDKPETLSSD
557	AKLVKHKKGIVNEQF
72	KPARAESKSFAVGMF

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, very long-chain specific acyl-CoA dehydrogenase acts specifically on fatty acyl-CoAs with saturated 12 to 24 carbons long primary chains, but can also catalyze monounsaturated fatty acids such as oleate ((9Z)-octadecenoate), (9Z)-hexadecenoate, and others. 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. Among the different mitochondrial acyl-CoA dehydrogenases, its FAO activity overlaps with that of ACAD9 and ACADL, but plays a primary role in tissues where it is the main long-chain ACAD expressed

Protein Sequence

10 MQSARMTPSV 20 GRQLLRLGAR 30 SSRSTTVLQG 40 QPRPISAQRL 50 YAREATQAVL 60 DKPETLSSDA 70 STREKPARAE 80 SKSFAVGMFK 90 GQLTIDQVFP 100 YPSVLSEEQA 110 QFLKELVGPV 120 ARFFEEVNDP 130 AKNDALEKVE 140 DDTLQGLKEL 150 GAFGLQVPSE 160 LGGLGLSNTQ 170 YARLAEIVGM 180 HDLGVSVTLG 190 AHQSIGFKGI 200 LLYGTKAQRE 210 KYLPRVASGQ 220 ALAAFCLTEP 230 SSGSDVASIR 240 SSAIPSPCGK 250 YYTLNGSKIW 260 ISNGGLADIF 270 TVFAKTPIKD 280 AATGAVKEKI 290 TAFVVERSFG 300 GVTHGLPEKK 310 MGIKASNTSE 320 VYFDGVKVPS 330 ENVLGEVGDG 340 FKVAVNILNN 350 GRFGMAATLA 360 GTMKSLIAKA 370 VDHATNRTQF 380 GDKIHNFGVI 390 QEKLARMAIL 400 QYVTESMAYM 410 LSANMDQGFK 420 DFQIEAAISK 430 IFCSEAAWKV 440 ADECIQIMGG 450 MGFMKEPGVE 460 RVLRDIRIFR 470 IFEGANDILR 480 LFVALQGCMD 490 KGKELTGLGN 500 ALKNPFGNVG 510 LLMGEAGKQL 520 RRRTGIGSGL 530 SLSGIVHPEL 540 SRSGELAVQA 550 LDQFATVVEA 560 KLVKHKKGIV 570 NEQFLLQRLA 580 DGAIDLYAMV 590 VVLSRASRSL 600 SEGYPTAQHE 610 KMLCDSWCIE 620 AATRIRENMA 630 SLQSSPQHQE 640 LFRNFRSISK 650 AMVENGGLVT GNPLGI

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005743	mitochondrial inner membrane
Cellular Component	GO:0031966	mitochondrial membrane
Cellular Component	GO:0042645	mitochondrial nucleoid
Cellular Component	GO:0005739	mitochondrion
Cellular Component	GO:0005730	nucleolus
Cellular Component	GO:0005654	nucleoplasm
Molecular Function	GO:0003995	acyl-CoA dehydrogenase activity
Molecular Function	GO:0000062	fatty-acyl-CoA binding
Molecular Function	GO:0050660	flavin adenine dinucleotide binding
Molecular Function	GO:0042802	identical protein binding
Molecular Function	GO:0004466	long-chain fatty acyl-CoA dehydrogenase activity
Molecular Function	GO:0017099	very-long-chain fatty acyl-CoA dehydrogenase activity
Biological Process	GO:0030855	epithelial cell differentiation
Biological Process	GO:0006635	fatty acid beta-oxidation
Biological Process	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase
Biological Process	GO:0009062	fatty acid catabolic process
Biological Process	GO:0045717	negative regulation of fatty acid biosynthetic process
Biological Process	GO:0046322	negative regulation of fatty acid oxidation
Biological Process	GO:0090181	regulation of cholesterol metabolic process
Biological Process	GO:0009409	response to cold
Biological Process	GO:0001659	temperature homeostasis

Reference

[1] Chang J, Wu W, Qian P, Lu Z, He X et al.. Multi-omics study on the effect of moderate-intensity exercise on protein lactylation in mouse muscle tissue.. Front Cell Dev Biol 12:1472338. 2024. PMID: 39935788.

[2] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[3] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.