PRODH2

proline dehydrogenase 2

Summary

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]

Known Variants46 total

rsidPosition (GRCh37)AllelesClassClinVar
rs133823953019:36,290,962C/Tuncertain significance
rs75226818719:36,290,975G/Auncertain significance
rs94251690319:36,291,014G/Auncertain significance
rs20208682819:36,291,022T/Auncertain significance
rs75948887919:36,291,034C/Tuncertain significance
rs37568386319:36,291,043T/Cuncertain significance
rs76235739219:36,291,047C/Auncertain significance
rs53889579619:36,291,048C/Guncertain significance
rs14690027419:36,291,052C/Tuncertain significance
rs76025691719:36,293,160A/Cuncertain significance
rs132549987419:36,293,175C/Tuncertain significance
rs76642321419:36,293,936T/Clikely benign
rs76772196419:36,293,951G/Auncertain significance
rs55335368819:36,293,972C/Tuncertain significance
rs37714659619:36,293,973G/Auncertain significance
rs7359441919:36,297,324G/Alikely benign
rs14351667219:36,297,395G/Auncertain significance
rs14627699619:36,297,423G/Tbenign
rs139140449619:36,297,464G/Tuncertain significance
rs14052491519:36,297,485C/Tuncertain significance
rs20126371419:36,297,486G/Auncertain significance
rs37486216719:36,297,488T/Auncertain significance
rs37067018319:36,297,620T/Cuncertain significance
rs18262811119:36,297,626G/Auncertain significance
rs77678018119:36,297,639G/Tuncertain significance
rs77235583719:36,297,710C/Tuncertain significance
rs14813900719:36,297,725C/Tlikely benign
rs7359442019:36,298,022T/Cbenign
rs20174236219:36,302,863C/Asplice region variant
rs11658313319:36,302,871G/Clikely benign
rs77161467819:36,302,911A/Tuncertain significance
rs2856303219:36,302,936C/Tbenign
rs77088111319:36,303,148C/Tuncertain significance
rs197262795419:36,303,313G/Auncertain significance
rs74688979719:36,303,400G/Auncertain significance
rs77097864419:36,303,403C/Tuncertain significance
rs14064952619:36,303,442C/Guncertain significance
rs14537071719:36,303,543G/Cuncertain significance
rs20179034519:36,303,656C/Guncertain significance
rs37020928719:36,303,700C/Guncertain significance
rs14797649319:36,303,701G/Auncertain significance
rs14180702419:36,303,736G/Alikely benign
rs54260634819:36,304,056T/Auncertain significance
rs6199573919:36,304,080G/Alikely benign
rs76905635219:36,304,112G/Alikely benign
rs75895576519:36,304,179G/Alikely benign

Gene information from NCBI Gene. Variant classifications from ClinVar.