Search Results

Overview

Uniprot IDP26440
Protein NameIsovaleryl-CoA dehydrogenase, mitochondrial
Gene NameIVD
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
262 ELIFEDCKIPAANIL
318 VREAFGQKIGHFQLM
401 GRFLRDAKLYEIGAG
58 QLRQTMAKFLQEHLA
67 LQEHLAPKAQEIDRS
78 IDRSNEFKNLREFWK

Function

A mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism, where isovaleryl-CoA (3-methylbutanoyl-CoA) is metabolized to 3-methylbut-2-enoyl-CoA (PubMed:7640268). To a lesser extent, it also participates in the first step in fatty acid beta-oxidation, in which it catalyzes the proR-proR stereospecific alpha,beta-dehydrogenation of other saturated short-chain acyl-CoA thioesters such as pentanoyl-CoA, hexanoyl-CoA and butanoyl-CoA, using the electron transfer flavoprotein (ETF) as their physiologic electron acceptor(PubMed:7640268)

Protein Sequence

10 MAEMATATRL 20 LGWRVASWRL 30 RPPLAGFVSQ 40 RAHSLLPVDD 50 AINGLSEEQR 60 QLRQTMAKFL 70 QEHLAPKAQE 80 IDRSNEFKNL 90 REFWKQLGNL 100 GVLGITAPVQ 110 YGGSGLGYLE 120 HVLVMEEISR 130 ASGAVGLSYG 140 AHSNLCINQL 150 VRNGNEAQKE 160 KYLPKLISGE 170 YIGALAMSEP 180 NAGSDVVSMK 190 LKAEKKGNHY 200 ILNGNKFWIT 210 NGPDADVLIV 220 YAKTDLAAVP 230 ASRGITAFIV 240 EKGMPGFSTS 250 KKLDKLGMRG 260 SNTCELIFED 270 CKIPAANILG 280 HENKGVYVLM 290 SGLDLERLVL 300 AGGPLGLMQA 310 VLDHTIPYLH 320 VREAFGQKIG 330 HFQLMQGKMA 340 DMYTRLMACR 350 QYVYNVAKAC 360 DEGHCTAKDC 370 AGVILYSAEC 380 ATQVALDGIQ 390 CFGGNGYIND 400 FPMGRFLRDA 410 KLYEIGAGTS 420 EVRRLVIGRA FNADFH

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0008470 3-methylbutanoyl-CoA dehydrogenase activity
Molecular Function GO:0050660 flavin adenine dinucleotide binding
Molecular Function GO:0042802 identical protein binding
Biological Process GO:0009083 branched-chain amino acid catabolic process
Biological Process GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase
Biological Process GO:0006552 L-leucine catabolic process

Reference

[1] Yang Z, Yan C, Ma J, Peng P, Ren X et al.. Lactylome analysis suggests lactylation-dependent mechanisms of metabolic adaptation in hepatocellular carcinoma.. Nat Metab 5(1):61-79. 2023 Jan. PMID: 36593272.

[2] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[3] Shi CM, Wang QC, Li XL, Yang YH, Tang XY et al.. Global Profiling of Protein Lactylation in Human Hippocampi.. Proteomics Clin Appl 19(2):e202400061. 2025 Mar. PMID: 39610256.