Reviewed, UniProtKB/Swiss-Prot Q8HXY4 (ODBA_MACFA)

Names and origin

Protein names

Recommended name:
    2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
    EC=1.2.4.4
Alternative name(s):
    Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
      Short name=BCKDH E1-alpha

Gene names

Name:	BCKDHA
ORF Names:	QccE-11424

Organism

Macaca fascicularis (Crab eating macaque) (Cynomolgus monkey)

Taxonomic identifier

9541 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Cercopithecidae › Cercopithecinae › Macaca

Protein attributes

Sequence length	445 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at transcript level.

General annotation (Comments)

Function	The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Catalytic activity	3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2.
Cofactor	Thiamine pyrophosphate By similarity.
Subunit structure	Heterotetramer of alpha and beta chains By similarity.
Subcellular location	Mitochondrion matrix By similarity.
Miscellaneous	Bound potassium ions stabilize the protein structure By similarity.
Sequence similarities	Belongs to the BCKDHA family.

Ontologies

Keywords
Cellular component	Mitochondrion
Domain	Transit peptide
Ligand	Metal-binding Potassium Thiamine pyrophosphate
Molecular function	Oxidoreductase
PTM	Phosphoprotein
Gene Ontology (GO)
Biological process	oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity Inferred from electronic annotation. Source: EC potassium ion binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 45	45	Mitochondrion By similarity
	Chain	46 – 445	400	2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial		PRO_0000020466
Regions
	Region	157 – 159	3	Thiamine pyrophosphate binding By similarity
Sites
	Metal binding	206	1	Potassium By similarity
	Metal binding	211	1	Potassium By similarity
	Metal binding	212	1	Potassium By similarity
Amino acid modifications
	Modified residue	337	1	Phosphoserine By similarity
	Modified residue	345	1	Phosphotyrosine By similarity
	Modified residue	347	1	Phosphoserine By similarity

Sequences

	Sequence		Length	Mass (Da)
	Q8HXY4-1 [UniParc]. Last modified March 1, 2003. Version 1. Checksum: AF645FF711238CA4	FASTA	445	50,538
	10 20 30 40 50 60 MAVAIAAARV WRPNRGLSQA ALLLLWRPGA RGLARSHPHR QQQQFSSLDD KPQFPGASAE 70 80 90 100 110 120 FIDKLEFIQP NVISGIPIYR VMDRQGQIIN PSEDPHLPKE KVLKLYKSMT LLNTMDRILY 130 140 150 160 170 180 ESQRQGRISF YMTNYGEEGT HVGSAAALDN TDLVFGQYRE AGVLMYRDYP LELFMAQCYG 190 200 210 220 230 240 NISDLGKGRQ MPVHYGCKER HFVTISSPLA TQIPQAVGAA YAAKRANANR VVICYFGEGA 250 260 270 280 290 300 ASEGDAHAGF NFAATLECPI IFFCRNNGYA ISTPTSEQYR GDGIAARGPG YGIMSIRVDG 310 320 330 340 350 360 NDVFAVYNAT KEARRRAVAE NQPFLIEAMT YRIGHHSTSD DSSAYRSVDE VNYWDKQDHP 370 380 390 400 410 420 ISRLRHYLLS QGWWDEEQEK AWRKQSRKKV MKAFEQAERK PKPNPNLLFS DVYQEMPAQL 430 440 RKQQESLARH LQTYGEHYPL EHFDK

References

[1]	"Isolation and characterization of cDNA for macaque neurological disease genes." Kusuda J., Osada N., Hida M., Sugano S., Hashimoto K. Submitted (APR-2002) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Brain cortex.

Cross-references

Sequence databases
EMBLGenBankDDBJ	AB083305 mRNA. Translation: BAC20584.1.
3D structure databases
HSSP	HSSP built from PDB template 1OLX based on UniProtKB P12694.
SMR	Q8HXY4. Positions 51-445.
ModBase	Search...
Phylogenomic databases
HOVERGEN	Q8HXY4.
Enzyme and pathway databases
BRENDA	1.2.4.4. 3438.
Family and domain databases
InterPro	IPR001017. DH_E1. [Graphical view]
Pfam	PF00676. E1_dh. 1 hit. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

ODBA_MACFA

Accession

Primary (citable) accession number: Q8HXY4

Entry history

Integrated into UniProtKB/Swiss-Prot:	April 12, 2005
Last sequence update:	March 1, 2003
Last modified:	June 16, 2009
This is version 40 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)