pyruvate dehydrogenase component x (dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex, E3-binding protein, E3BP, proX, PDHX, PDX1)

Function

• required for anchoring dihydrolipoamide dehydrogenase E3 to the dihydrolipoamide transacetylase E2 core of the pyruvate dehydrogenase complexes of eukaryotes; this binding is essential for a functional PDH complex

Structure

• belongs to the 2-oxoacid dehydrogenase family
• contains 1 lipoyl-binding domain

Compartment

mitochondrial matrix

Pathology

• defects in PDHX are a cause of congenital infantile lactic acidosis

More general terms

• protein subunit
• mitochondrial protein

Component of

• pyruvate dehydrogenase

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:8050
• OMIM: https://mirror.omim.org/entry/245349
• OMIM: https://mirror.omim.org/entry/608769
• UniProt: http://www.uniprot.org/uniprot/O00330.html