The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/50 - 1/100.
1/100 - 1/500. Detects a band of approximately 54 kDa (predicted molecular weight: 54 kDa).
Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
Involvement in disease
Defects in PDHX are the cause of pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349].
Belongs to the 2-oxoacid dehydrogenase family. Contains 1 lipoyl-binding domain.