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Overview

Uniprot IDA0A8I6A3D0
Protein NameIsovaleryl-CoA dehydrogenase, mitochondrial
Gene NameIvd
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
239 MPGFSTSKKLDKLGM
56 QLRHTISKFVQENLA
76 IDQSNDFKNLREFWK

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. 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

Protein Sequence

10 MATAVRLLGR 20 RVSSWRLRPL 30 PSPLAVPQRA 40 HSMLPVDDDI 50 NGLNEEQKQL 60 RHTISKFVQE 70 NLAPKAQEID 80 QSNDFKNLRE 90 FWKQLGSLGV 100 LGITAPVQYG 110 GSGLGYLEHV 120 LVMEEISRAS 130 AAVGLSYGAH 140 SNLCINQIVR 150 NGNEAQKEKY 160 LPKLISGEFI 170 GALAMSEPNA 180 GSDVVSMRLK 190 AEKKGDHYVL 200 NGNKFWITNG 210 PDADVLVVYA 220 KTDLTAVPAS 230 RGITAFIVEK 240 DMPGFSTSKK 250 LDKLGMRGSN 260 TCELVFEDCK 270 VPAANILSQE 280 SKGVYVLMSG 290 LDLERLVLAG 300 GPLGIMQAVL 310 DHTIPYLHVR 320 EAFGQKIGQF 330 QLMQGKMADM 340 YTRLMACRQY 350 VYNVARACGN 360 GYINDFPMGR 370 FLRDAKLYEI 380 GGGTSEVRRL 390 VIGRAFNADF R

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0008470 3-methylbutanoyl-CoA dehydrogenase activity
Molecular Function GO:0050660 flavin adenine dinucleotide binding
Biological Process GO:0006552 L-leucine catabolic process

Reference

[1] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.