Search Results

Overview

Uniprot IDQ6NVY1
Protein Name3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Gene NameHIBCH
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
112 EAEKAKQKIAPVFFR
237 LALKSPSKENIASVL
252 ENYHTESKIDRDKSF
257 ESKIDRDKSFILEEH
301 EQLKVINKMSPTSLK
353 VRAVLIDKDQSPKWK
73 RQIYPQLKKWEQDPE
92 IIKGAGGKAFCAGGD

Function

Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA

Protein Sequence

10 MGQREMWRLM 20 SRFNAFKRTN 30 TILHHLRMSK 40 HTDAAEEVLL 50 EKKGCTGVIT 60 LNRPKFLNAL 70 TLNMIRQIYP 80 QLKKWEQDPE 90 TFLIIIKGAG 100 GKAFCAGGDI 110 RVISEAEKAK 120 QKIAPVFFRE 130 EYMLNNAVGS 140 CQKPYVALIH 150 GITMGGGVGL 160 SVHGQFRVAT 170 EKCLFAMPET 180 AIGLFPDVGG 190 GYFLPRLQGK 200 LGYFLALTGF 210 RLKGRDVYRA 220 GIATHFVDSE 230 KLAMLEEDLL 240 ALKSPSKENI 250 ASVLENYHTE 260 SKIDRDKSFI 270 LEEHMDKINS 280 CFSANTVEEI 290 IENLQQDGSS 300 FALEQLKVIN 310 KMSPTSLKIT 320 LRQLMEGSSK 330 TLQEVLTMEY 340 RLSQACMRGH 350 DFHEGVRAVL 360 IDKDQSPKWK 370 PADLKEVTEE 380 DLNNHFKSLG SSDLKF

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0003860 3-hydroxyisobutyryl-CoA hydrolase activity
Biological Process GO:0009083 branched-chain amino acid catabolic process
Biological Process GO:0006574 L-valine 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.