Cannabis Compound Database: Showing Protein 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (CDBP00293)

Identification Biological properties Gene properties Protein properties External links References XML Show 11 compounds

Identification

HMDB Protein ID

CDBP00293

Secondary Accession Numbers

Not Available

Name

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

Description

Not Available

Synonyms

BCKDE1B
BCKDH E1-beta
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain

Gene Name

BCKDHB

Protein Type

Enzyme

Biological Properties

General Function

Involved in catalytic activity

Specific Function

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).

GO Classification

Biological Process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
response to nutrient
response to glucocorticoid stimulus
response to cAMP
Cellular Component
mitochondrial alpha-ketoglutarate dehydrogenase complex
Function
catalytic activity
Molecular Function
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
alpha-ketoacid dehydrogenase activity
carboxy-lyase activity
Process
metabolic process

Cellular Location

Mitochondrion matrix

Pathways

Name	SMPDB/Pathwhiz	KEGG
Valine, Leucine and Isoleucine Degradation
Beta-Ketothiolase Deficiency		Not Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency		Not Available
Propionic Acidemia		Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency		Not Available

Gene Properties

Chromosome Location

Locus

6q14.1

SNPs

BCKDHB

Gene Sequence

>1179 bp
ATGGCGGTTGTAGCGGCGGCTGCCGGCTGGCTACTCAGGCTCAGGGCGGCAGGGGCTGAG
GGGCACTGGCGTCGGCTTCCTGGCGCGGGGCTGGCGCGGGGCTTTTTGCACCCCGCCGCG
ACTGTCGAGGATGCGGCCCAGAGGCGGCAGGTGGCTCATTTTACTTTCCAGCCAGATCCG
GAGCCCCGGGAGTACGGGCAAACTCAGAAAATGAATCTTTTCCAGTCTGTAACAAGTGCC
TTGGATAACTCATTGGCCAAAGATCCTACTGCAGTAATATTTGGTGAAGATGTTGCCTTT
GGTGGAGTCTTTAGATGCACTGTTGGCTTGCGAGACAAATATGGAAAAGATAGAGTTTTT
AATACCCCATTGTGTGAACAAGGAATTGTTGGATTTGGAATCGGAATTGCGGTCACTGGA
GCTACTGCCATTGCGGAAATTCAGTTTGCAGATTATATTTTCCCTGCATTTGATCAGATT
GTTAATGAAGCTGCCAAGTATCGCTATCGCTCTGGGGATCTTTTTAACTGTGGAAGCCTC
ACTATCCGGTCCCCTTGGGGCTGTGTTGGTCATGGGGCTCTCTATCATTCTCAGAGTCCT
GAAGCATTTTTTGCCCATTGCCCAGGAATCAAGGTGGTTATACCCAGAAGCCCTTTCCAG
GCCAAAGGACTTCTTTTGTCATGCATAGAGGATAAAAATCCTTGTATATTTTTTGAACCT
AAAATACTTTACAGGGCAGCAGCGGAAGAAGTCCCTATAGAACCATACAACATCCCACTG
TCCCAGGCCGAAGTCATACAGGAAGGGAGTGATGTTACTCTAGTTGCCTGGGGCACTCAG
GTTCATGTGATCCGAGAGGTAGCTTCCATGGCAAAAGAAAAGCTTGGAGTGTCTTGTGAA
GTCATTGATCTGAGGACTATAATACCTTGGGATGTGGACACAATTTGTAAGTCTGTGATC
AAAACAGGGCGACTGCTAATCAGTCACGAGGCTCCCTTGACAGGCGGCTTTGCATCGGAA
ATCAGCTCTACAGTTCAGGAGGAATGTTTCTTGAACCTAGAGGCTCCTATATCAAGAGTA
TGTGGTTATGACACACCATTTCCTCACATTTTTGAACCATTCTACATCCCAGACAAATGG
AAGTGTTATGATGCCCTTCGAAAAATGATCAACTATTGA

Protein Properties

Number of Residues

392

Molecular Weight

43122.065

Theoretical pI

6.293

Pfam Domain Function

Transket_pyr (PF02779 )
Transketolase_C (PF02780 )

Signals

Not Available

Transmembrane Regions

Not Available

Protein Sequence

>2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDP
EPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVF
NTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSL
TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEP
KILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCE
VIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRV
CGYDTPFPHIFEPFYIPDKWKCYDALRKMINY

External Links

GenBank ID Protein

179362

UniProtKB/Swiss-Prot ID

P21953

UniProtKB/Swiss-Prot Entry Name

ODBB_HUMAN

PDB IDs