PYCR2

Chr 1AR

pyrroline-5-carboxylate reductase 2

Also known as: HLD10, P5CR2

This gene belongs to the pyrroline-5-carboxylate reductase family. The encoded mitochondrial protein catalyzes the conversion of pyrroline-5-carboxylate to proline, which is the last step in proline biosynthesis. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Nov 2012]

OMIMResearchGenerating clinical summary…
MultiplemechanismARLOEUF 1.341 OMIM phenotype
Clinical SummaryPYCR2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.34LOEUF
pLI 0.000
Z-score 0.69
OE 0.79 (0.491.34)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.95Z-score
OE missense 0.81 (0.710.92)
156 obs / 193.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.79 (0.491.34)
00.351.4
Missense OE?0.81 (0.710.92)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 10 / 12.6Missense obs/exp: 156 / 193.1Syn Z: -0.52
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongPYCR2-related postnatal microcephaly, hypomyelination, and reduced cerebral white-matter volumeOTHERAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.75top 25%
GOF
0.6442th %ile
LOF
0.3259th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PYCR2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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