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Overview

Uniprot ID	P12694
Protein Name	2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Gene Name	BCKDHA
Organism	Homo sapiens

Kla Sites from experimental identification

Position	Flanking peptide
389	WRKQSRRKVMEAFEQ

Function

Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction

Protein Sequence

10 MAVAIAAARV 20 WRLNRGLSQA 30 ALLLLRQPGA 40 RGLARSHPPR 50 QQQQFSSLDD 60 KPQFPGASAE 70 FIDKLEFIQP 80 NVISGIPIYR 90 VMDRQGQIIN 100 PSEDPHLPKE 110 KVLKLYKSMT 120 LLNTMDRILY 130 ESQRQGRISF 140 YMTNYGEEGT 150 HVGSAAALDN 160 TDLVFGQYRE 170 AGVLMYRDYP 180 LELFMAQCYG 190 NISDLGKGRQ 200 MPVHYGCKER 210 HFVTISSPLA 220 TQIPQAVGAA 230 YAAKRANANR 240 VVICYFGEGA 250 ASEGDAHAGF 260 NFAATLECPI 270 IFFCRNNGYA 280 ISTPTSEQYR 290 GDGIAARGPG 300 YGIMSIRVDG 310 NDVFAVYNAT 320 KEARRRAVAE 330 NQPFLIEAMT 340 YRIGHHSTSD 350 DSSAYRSVDE 360 VNYWDKQDHP 370 ISRLRHYLLS 380 QGWWDEEQEK 390 AWRKQSRRKV 400 MEAFEQAERK 410 PKPNPNLLFS 420 DVYQEMPAQL 430 RKQQESLARH 440 LQTYGEHYPL DHFDK

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0160157	branched-chain alpha-ketoacid dehydrogenase complex
Cellular Component	GO:0005759	mitochondrial matrix
Cellular Component	GO:0005739	mitochondrion
Molecular Function	GO:0003863	branched-chain 2-oxo acid dehydrogenase activity
Molecular Function	GO:0016831	carboxy-lyase activity
Molecular Function	GO:0046872	metal ion binding
Biological Process	GO:0120552	branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA
Biological Process	GO:0009083	branched-chain amino acid 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.