PDPR
Description
The PDPR (pyruvate dehydrogenase phosphatase regulatory subunit) is a protein-coding gene located on chromosome 16.
The PDPR gene encodes a protein known as pyruvate dehydrogenase phosphatase regulatory subunit. This protein is involved in the regulation of the pyruvate dehydrogenase complex (PDC), a crucial enzyme in cellular metabolism. PDC catalyzes the conversion of pyruvate to acetyl-CoA, linking glycolysis to the tricarboxylic acid cycle. PDPR acts as a regulatory subunit of the pyruvate dehydrogenase phosphatase (PDP), which activates PDC by removing phosphate groups. PDPR decreases the sensitivity of PDP1 to magnesium ions, and this inhibition is reversed by the polyamine spermine. PDPR shares distant homology with dimethylglycine dehydrogenase, a mitochondrial flavoprotein involved in choline degradation. While PDPR's role in disease pathophysiology is likely due to its involvement in a central metabolic pathway, further research is needed to elucidate its specific contributions.
PDPR is also known as PDP3.
Associated Diseases
- Alzheimer disease
- Parkinson disease
- multiple sclerosis
- lysosomal storage disease
- diabetes mellitus, permanent neonatal 4
- diabetes mellitus, transient neonatal, 3
- permanent neonatal diabetes mellitus 1
- X-linked sideroblastic anemia 1
