PRODH2

The protein encoded by this gene catalyzes the first step in the catabolism of trans-4-hydroxy-L-proline, an amino acid derivative obtained through food intake and collagen turnover. One of the downstream products of this catabolism is glyoxylate, which in people with disorders of glyoxalate metabolism can lead to an increase in oxalate levels and the formation of calcium-oxalate kidney stones. Therefore, this gene may serve as a therapeutic target against primary hyperoxalurias (PH). This gene is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme that catalyzes the first step in proline catabolism. [provided by RefSeq, Jan 2017]

Description

Proline dehydrogenase 2

Tissue specificity

Group enriched (kidney, liver)

Subcell location

Nucleoplasm, Cytosol

Predicted location

Intracellular

Protein family

Enzymes
Mapped to neXtProt
Mapped to UniProt SWISS-PROT
Metabolic proteins
Predicted intracellular proteins
Protein evidence (Ezkurdia et al 2014)

Chromosome

19 (35799988 - 35813299)

Ensembl ID

ENSG00000250799  (ver. 103.38)

Orthologs

Mouse Prodh2: ENSMUSG00000036892

UniProt

Q9UF12

neXtProt

NX_Q9UF12

Human splice variants
Mouse splice variants

Protein structure