ID   ODBA_MOUSE              Reviewed;         442 AA.
AC   P50136;
DT   01-OCT-1996, integrated into UniProtKB/Swiss-Prot.
DT   01-OCT-1996, sequence version 1.
DT   25-NOV-2008, entry version 74.
DE   RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;
DE            EC=1.2.4.4;
DE   AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;
DE            Short=BCKDH E1-alpha;
DE   Flags: Precursor;
GN   Name=Bckdha;
OS   Mus musculus (Mouse).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
OC   Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Sciurognathi;
OC   Muroidea; Muridae; Murinae; Mus.
OX   NCBI_TaxID=10090;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [MRNA].
RC   STRAIN=C57BL/6J; TISSUE=Liver;
RX   MEDLINE=96358490; PubMed=8761456;
RA   Costeas P.A., Chinsky J.M.;
RT   "Effects of insulin on the regulation of branched-chain alpha-keto
RT   acid dehydrogenase E1 alpha subunit gene expression.";
RL   Biochem. J. 318:85-92(1996).
RN   [2]
RP   PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-344, AND MASS
RP   SPECTROMETRY.
RC   TISSUE=Liver;
RX   PubMed=17203969; DOI=10.1021/pr0604155;
RA   Dai J., Jin W.-H., Sheng Q.-H., Shieh C.-H., Wu J.-R., Zeng R.;
RT   "Protein phosphorylation and expression profiling by Yin-yang
RT   multidimensional liquid chromatography (Yin-yang MDLC) mass
RT   spectrometry.";
RL   J. Proteome Res. 6:250-262(2007).
RN   [3]
RP   PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-334, AND MASS
RP   SPECTROMETRY.
RC   TISSUE=Liver;
RX   PubMed=17208939; DOI=10.1074/mcp.M600218-MCP200;
RA   Lee J., Xu Y., Chen Y., Sprung R., Kim S.C., Xie S., Zhao Y.;
RT   "Mitochondrial phosphoproteome revealed by an improved IMAC method and
RT   MS/MS/MS.";
RL   Mol. Cell. Proteomics 6:669-676(2007).
RN   [4]
RP   PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-344, AND MASS
RP   SPECTROMETRY.
RC   TISSUE=Liver;
RX   PubMed=17242355; DOI=10.1073/pnas.0609836104;
RA   Villen J., Beausoleil S.A., Gerber S.A., Gygi S.P.;
RT   "Large-scale phosphorylation analysis of mouse liver.";
RL   Proc. Natl. Acad. Sci. U.S.A. 104:1488-1493(2007).
CC   -!- FUNCTION: The branched-chain alpha-keto dehydrogenase complex
CC       catalyzes the overall conversion of alpha-keto acids to acyl-CoA
CC       and CO(2). It contains multiple copies of three enzymatic
CC       components: branched-chain alpha-keto acid decarboxylase (E1),
CC       lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
CC   -!- CATALYTIC ACTIVITY: 3-methyl-2-oxobutanoate +
CC       [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
CC       lipoyllysine = [dihydrolipoyllysine-residue (2-
CC       methylpropanoyl)transferase] S-(2-
CC       methylpropanoyl)dihydrolipoyllysine + CO(2).
CC   -!- COFACTOR: Thiamine pyrophosphate.
CC   -!- SUBUNIT: Heterotetramer of alpha and beta chains (By similarity).
CC   -!- SUBCELLULAR LOCATION: Mitochondrion matrix.
CC   -!- MISCELLANEOUS: Bound potassium ions stabilize the protein
CC       structure (By similarity).
CC   -!- SIMILARITY: Belongs to the BCKDHA family.
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DR   EMBL; L47335; AAB38422.1; -; mRNA.
DR   PIR; S71881; S71881.
DR   UniGene; Mm.25848; -.
DR   HSSP; P12694; 1DTW.
DR   SMR; P50136; 48-442.
DR   SWISS-2DPAGE; P50136; -.
DR   Ensembl; ENSMUSG00000060376; Mus musculus.
DR   MGI; MGI:107701; Bckdha.
DR   HOVERGEN; P50136; -.
DR   GermOnline; ENSMUSG00000060376; Mus musculus.
DR   GO; GO:0005947; C:mitochondrial alpha-ketoglutarate dehydroge...; ISS:HGNC.
DR   GO; GO:0003863; F:3-methyl-2-oxobutanoate dehydrogenase (2-me...; IEA:EC.
DR   GO; GO:0003826; F:alpha-ketoacid dehydrogenase activity; ISS:HGNC.
DR   GO; GO:0016536; F:cyclin-dependent protein kinase 5 activator...; IDA:MGI.
DR   GO; GO:0030955; F:potassium ion binding; IEA:UniProtKB-KW.
DR   GO; GO:0009083; P:branched chain family amino acid catabolic ...; ISS:HGNC.
DR   GO; GO:0055114; P:oxidation reduction; IEA:UniProtKB-KW.
DR   GO; GO:0045893; P:positive regulation of transcription, DNA-d...; IDA:MGI.
DR   InterPro; IPR001017; DHase_E1.
DR   Pfam; PF00676; E1_dh; 1.
PE   1: Evidence at protein level;
KW   Metal-binding; Mitochondrion; Oxidoreductase; Phosphoprotein;
KW   Potassium; Thiamine pyrophosphate; Transit peptide.
FT   TRANSIT       1     42       Mitochondrion (By similarity).
FT   CHAIN        43    442       2-oxoisovalerate dehydrogenase subunit
FT                                alpha, mitochondrial.
FT                                /FTId=PRO_0000020467.
FT   REGION      154    156       Thiamine pyrophosphate binding (By
FT                                similarity).
FT   METAL       203    203       Potassium (By similarity).
FT   METAL       208    208       Potassium (By similarity).
FT   METAL       209    209       Potassium (By similarity).
FT   MOD_RES     334    334       Phosphoserine.
FT   MOD_RES     342    342       Phosphotyrosine (By similarity).
FT   MOD_RES     344    344       Phosphoserine.
SQ   SEQUENCE   442 AA;  50371 MW;  3388213D88BC7C92 CRC64;
     MSAAKIWRPS RGLRQAALLL LGRSGVRGLA RSHPSRQQQQ QFPSLDDKPQ FPGASAEFVD
     KLEFIQPNVI SGIPIYRVMD RQGQIINPSE DPHLPQEEVL KFYRSMTLLN TMDRILYESQ
     REGRISFYMT NYGEEGTHVG SAAALERTDL VFGQYREAGV LMYRDYPLEL FMSQCYGNVN
     DPGKGRQMPV HYGCKERHFV TISSPLATQI PQAVGAAYAA KRANANRIVI CYFGEGAASE
     GDAHAGFNFA ATLECPIIFF CRNNGYAIST PTSEQYRGDG IAARGPGYGI KSIRVDGNDV
     FAVYNATKEA RRRAVAENQP FLIEAMTYRI GHHSTSDDSS AYRSVDEVNY WDKQDHPISR
     LRQYLLNQGW WDEEQEKAWR KQSRKKVMEA FEQAERKLKP NPSLLFSDVY QEMPAQLRRQ
     QESLARHLQT YGEHYPLDHF EK
//