PRODH2

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Description

The PRODH2 (proline dehydrogenase 2) is a protein-coding gene located on chromosome 19.

PRODH2 is an enzyme that converts trans-4-L-hydroxyproline to delta-1-pyrroline-3-hydroxy-5-carboxylate (Hyp) using ubiquinone-10 as the electron acceptor. While it can also utilize proline as a substrate, its efficiency is significantly lower. PRODH2 does not interact with other diastereomers of Hyp, such as trans-4-D-hydroxyproline and cis-4-L-hydroxyproline. Notably, PRODH2 exhibits higher efficiency when utilizing ubiquinone analogs like menadione, duroquinone, and ubiquinone-1 as terminal electron acceptors compared to oxygen.

PRODH2 is also known as HSPOX1, HYPDH.

Associated Diseases

• seizures-intellectual disability due to hydroxylysinuria syndrome
• hyperdibasic aminoaciduria type 1
• carnosinemia
• cystinuria
• iminoglycinuria
• hyperprolinemia type 2
• primary hyperoxaluria type 3
• saccharopinuria
• hyperprolinemia type 1
• primary hyperoxaluria type 2
• pentosuria
• phosphohydroxylysinuria
• beta-aminoisobutyric acid, urinary excretion of
• cystathioninuria
• Dent disease
• histidinemia
• dicarboxylic aminoaciduria
• Fanconi renotubular syndrome 1
• camptodactyly-taurinuria syndrome
• autosomal dominant progressive nephropathy with hypertension
• tyrosinemia type II