CPLM-008253

Genbank Nucleotide ID

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Protein Synonyms/Alias

Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; BCKDE1A; BCKDH E1-alpha

Mus musculus (Mouse)

Position	Peptide	Type	References
101	LPQEEVLKFYRSMTL	acetylation	[1]
308	FAVYNATKEARRRAV	acetylation	[1, 2]
353	DEVNYWDKQDHPISR	acetylation	[1, 2, 3, 4, 5, 6]
353	DEVNYWDKQDHPISR	succinylation	[6]
353	DEVNYWDKQDHPISR	ubiquitination	[7]
377	WWDEEQEKAWRKQSR	acetylation	[1, 2, 4, 6]
377	WWDEEQEKAWRKQSR	succinylation	[6]
386	WRKQSRKKVMEAFEQ	acetylation	[2, 6]
386	WRKQSRKKVMEAFEQ	succinylation	[6]

[1] Quantification of mitochondrial acetylation dynamics highlights prominent sites of metabolic regulation.
Still AJ, Floyd BJ, Hebert AS, Bingman CA, Carson JJ, Gunderson DR, Dolan BK, Grimsrud PA, Dittenhafer-Reed KE, Stapleton DS, Keller MP, Westphall MS, Denu JM, Attie AD, Coon JJ, Pagliarini DJ.
J Biol Chem. 2013 Jul 17;. [PMID: 23864654]
[2] Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.
Rardin MJ, Newman JC, Held JM, Cusack MP, Sorensen DJ, Li B, Schilling B, Mooney SD, Kahn CR, Verdin E, Gibson BW.
Proc Natl Acad Sci U S A. 2013 Apr 16;110(16):6601-6. [PMID: 23576753]
[3] The fasted/fed mouse metabolic acetylome: N6-acetylation differences suggest acetylation coordinates organ-specific fuel switching.
Yang L, Vaitheesvaran B, Hartil K, Robinson AJ, Hoopmann MR, Eng JK, Kurland IJ, Bruce JE.
J Proteome Res. 2011 Sep 2;10(9):4134-49. [PMID: 21728379]
[4] Quantitative assessment of the impact of the gut microbiota on lysine epsilon-acetylation of host proteins using gnotobiotic mice.
Simon GM, Cheng J, Gordon JI.
Proc Natl Acad Sci U S A. 2012 Jul 10;109(28):11133-8. [PMID: 22733758]
[5] Circadian acetylome reveals regulation of mitochondrial metabolic pathways.
Masri S, Patel VR, Eckel-Mahan KL, Peleg S, Forne I, Ladurner AG, Baldi P, Imhof A, Sassone-Corsi P.
Proc Natl Acad Sci U S A. 2013 Feb 26;110(9):3339-44. [PMID: 23341599]
[6] SIRT5-Mediated Lysine Desuccinylation Impacts Diverse Metabolic Pathways.
Park J, Chen Y, Tishkoff DX, Peng C, Tan M, Dai L, Xie Z, Zhang Y, Zwaans BM, Skinner ME, Lombard DB, Zhao Y.
Mol Cell. 2013 Jun 27;50(6):919-30. [PMID: 23806337]
[7] Proteomic analyses reveal divergent ubiquitylation site patterns in murine tissues.
Wagner SA, Beli P, Weinert BT, Schölz C, Kelstrup CD, Young C, Nielsen ML, Olsen JV, Brakebusch C, Choudhary C.
Mol Cell Proteomics. 2012 Dec;11(12):1578-85. [PMID: 22790023]

Functional Description

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).

REGION 154 156 Thiamine pyrophosphate binding (By
METAL 203 203 Potassium (By similarity).
METAL 208 208 Potassium (By similarity).
METAL 209 209 Potassium (By similarity).
MOD_RES 334 334 Phosphoserine.
MOD_RES 344 344 Phosphoserine.

Complete proteome; Metal-binding; Mitochondrion; Oxidoreductase; Phosphoprotein; Potassium; Reference proteome; Thiamine pyrophosphate; Transit peptide.

UniProt (SWISSPROT/TrEMBL); GenBank; EMBL

GO:0005947; C:mitochondrial alpha-ketoglutarate dehydrogenase complex; ISS:HGNC.
GO:0003863; F:3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity; IEA:EC.
GO:0003826; F:alpha-ketoacid dehydrogenase activity; ISS:HGNC.
GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
GO:0009083; P:branched-chain amino acid catabolic process; ISS:HGNC.