InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PDHB

Summary

InnateDB Protein

IDBP-42229.7

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PDHB

Protein Name

pyruvate dehydrogenase (lipoamide) beta

Synonyms

PDHBD; PDHE1-B; PHE1B;

Species

Homo sapiens

Ensembl Protein

ENSP00000373220

InnateDB Gene

IDBG-42225 (PDHB)

Protein Structure

UniProt Annotation

Function

The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. {ECO:0000269PubMed:17474719, ECO:0000269PubMed:19081061}.

Subcellular Localization

Mitochondrion matrix.

Disease Associations

Pyruvate dehydrogenase E1-beta deficiency (PDHBD) [MIM:614111]: An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis. {ECO:0000269PubMed:15138885}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 48 experimentally validated interaction(s) in this database.
They are also associated with 2 interaction(s) predicted by orthology.

Experimentally validated
Total	48 [view]
Protein-Protein	48 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	2 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004738	pyruvate dehydrogenase activity
GO:0004739	pyruvate dehydrogenase (acetyl-transferring) activity
GO:0005515	protein binding

Biological Process

GO:0006006	glucose metabolic process
GO:0006086	acetyl-CoA biosynthetic process from pyruvate
GO:0006090	pyruvate metabolic process
GO:0006099	tricarboxylic acid cycle
GO:0008152	metabolic process
GO:0010510	regulation of acetyl-CoA biosynthetic process from pyruvate
GO:0044237	cellular metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0005634	nucleus
GO:0005759	mitochondrial matrix
GO:0045254	pyruvate dehydrogenase complex
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR005475 Transketolase-like, pyrimidine-binding domain
IPR005476 Transketolase, C-terminal
IPR009014 Transketolase, C-terminal/Pyruvate-ferredoxin oxidoreductase, domain II
IPR029061 Thiamin diphosphate-binding fold

PFAM

PF02779
PF02780

PRINTS

PIRSF

SMART

SM00861

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

P11177