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Overview

Uniprot IDA0A8I6AP13
Protein NameTrifunctional enzyme subunit beta, mitochondrial
Gene NameHadhb
OrganismRattus norvegicus

Kla Sites from experimental identification

Position Flanking peptide
189 KMMLDLNKAKTLAQR
244 VSRMEQDKYALRSHS
273 VPFKVPGKDTVSKDN

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 QLLLGPTYAT 370 PKVLEKAGLT 380 MNDIDAFEFH 390 EAFSGQILAN 400 FKAMDSDWFA 410 QNYMGRKTKV 420 GAPPLEKFNI 430 WGGSLSLGHP 440 FGATGCRLVM 450 AAANRLRKDG 460 GQYALVAACA 470 AGGQVRHCTI

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005739 mitochondrion
Molecular Function GO:0003988 acetyl-CoA C-acyltransferase activity
Molecular Function GO:0050633 acetyl-CoA C-myristoyltransferase activity
Biological Process GO:0006631 fatty acid metabolic 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.