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Overview

Uniprot IDA0A0G2K330
Protein NameTrifunctional enzyme subunit beta, mitochondrial
Gene NameHadhb
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
189 KMMLDLNKAKTLAQR
244 VSRMEQDKYALRSHS
273 VPFKVPGKDTVSKDN
371 RPTYATPKVLEKAGL

Function

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity

Protein Sequence

10 MTTILTSTFR 20 NLSTTSKWAL 30 RFSVRPLSCS 40 SQVQSAPAVQ 50 TKSKKTLAKP 60 NLKNIVVVEG 70 VRIPFLLSGT 80 SYKDLMPHDL 90 ARAALSGLLY 100 RTNIPKDVVD 110 YIIFGTVIQE 120 VKTSNVAREA 130 ALGAGFSDKT 140 PAHTVTMACI 150 SSNQAMTTAV 160 GLIASGQCDV 170 VVAGGVELMS 180 DVPIRHSRNM 190 RKMMLDLNKA 200 KTLAQRLSLL 210 TKFRLNFLSP 220 ELPAVAEFST 230 NETMGHSADR 240 LAAAFAVSRM 250 EQDKYALRSH 260 SLAKKAQDEG 270 HLSDIVPFKV 280 PGKDTVSKDN 290 GIRPSSLEQM 300 AKLKPAFIKP 310 YGTVTAANSS 320 FLTDGASAML 330 IMSEDRALAM 340 GYKPKAYLRD 350 FIYVSQDPKD 360 QLLLGDQLRS 370 FLHRPTYATP 380 KVLEKAGLTM 390 NDIDAFEFHE 400 AFSGQILANF 410 KAMDSDWFAQ 420 NYMGRKTKVG 430 APPLEKFNIW 440 GGSLSLGHPF 450 GATGCRLVMA 460 AANRLRKDGG 470 QYALVAACAA 480 GGQGHAMIVE AYPK

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005783 endoplasmic reticulum
Cellular Component GO:0016507 mitochondrial fatty acid beta-oxidation multienzyme complex
Cellular Component GO:0005743 mitochondrial inner membrane
Cellular Component GO:0005741 mitochondrial outer membrane
Molecular Function GO:0003988 acetyl-CoA C-acyltransferase activity
Molecular Function GO:0050633 acetyl-CoA C-myristoyltransferase activity
Biological Process GO:0006635 fatty acid beta-oxidation

Reference

[1] Yao Y, Bade R, Li G, Zhang A, Zhao H et al.. Global-Scale Profiling of Differential Expressed Lysine-Lactylated Proteins in the Cerebral Endothelium of Cerebral Ischemia-Reperfusion Injury Rats.. Cell Mol Neurobiol 43(5):1989-2004. 2023 Jul. PMID: 36030297.

[2] Sheng L, Xu H, Wang Y, Ni J, Xiang T et al.. Systematic analysis of lysine lactylation in nucleus pulposus cells.. iScience 27(11):111157. 2024 Nov 15. PMID: 39524337.

[3] 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.