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Overview

Uniprot IDA0A5G2QGJ8
Protein NameTrifunctional enzyme subunit beta, mitochondrial
Gene NameHADHB
OrganismSus scrofa

Kla Sites from experimental identification

Position Flanking peptide
166 QRLSLISKFRLNFLS
373 NYMGRKAKVGSPPLE

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 YSVQTKSKKT 20 LAKPNIRNIV 30 VVDGVRIPFL 40 QSGTSYKELM 50 PHDLARAALL 60 GLLHRTSVPK 70 DVVDYLVFGT 80 VIQEVKTSNV 90 AREAALGAGF 100 SDKTPAHTVT 110 MACISSNQAM 120 TTGVSLIASG 130 QFDVVVAGGV 140 ELMSDIPIRH 150 SRKMRKMMLD 160 LNKAKTLGQR 170 LSLISKFRLN 180 FLSPELPAVA 190 EFSTSETMGH 200 SADRLAAAFA 210 VSRSEQDEYA 220 LRSHSLAKKA 230 QDEGLLSDVV 240 PFKVPGKDTV 250 TQDNGIRPSS 260 LEQMAKLKPA 270 FIKPYGTVTA 280 ANSSFLTDGA 290 SAVLIMAEEK 300 ALAMGYKPKA 310 YLRDFTYVSQ 320 DPKDQLLLGP 330 TYATPKVLEK 340 AGLTMNDIDA 350 FEFHEAFSGQ 360 ILANLKAMDS 370 DWFAQNYMGR 380 KAKVGSPPLE 390 KFNNWGGSLS 400 LGHPFGATGC 410 RLVMTAANRL 420 RKEGGQYGLV 430 AACAAGGQVL 440 IHIYDESYEK YKKYVVKN

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] Fan S, Zhou R, Wen H, Ye H, Ma S et al.. Global profiling of protein lactylome in porcine granulosa cells.. J Ovarian Res 18(1):177. 2025 Aug 8. PMID: 40781717.