PRXL2B

Chr 1

peroxiredoxin like 2B

Also known as: C1orf93, FAM213B, PM/PGFS

The protein catalyzes the reduction of prostaglandin H2 to prostaglandin F2alpha and reduces prostamide H2 to prostamide F2alpha using NADPH as a proton donor. The gene is not currently associated with any established Mendelian diseases in pediatric neurology. This gene shows low constraint to loss-of-function variation, suggesting tolerance to functional disruption.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
125
P/LP submissions
0%
P/LP missense
1.52
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryPRXL2B
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
118 unique Pathogenic / Likely Pathogenic· 67 VUS of 208 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.52LOEUF
pLI 0.000
Z-score 0.43
OE 0.85 (0.501.52)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.35Z-score
OE missense 0.90 (0.771.07)
97 obs / 107.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.85 (0.501.52)
00.351.4
Missense OE0.90 (0.771.07)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 8 / 9.4Missense obs/exp: 97 / 107.3Syn Z: 0.12
DN
0.6260th %ile
GOF
0.72top 25%
LOF
0.2287th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

208 submitted variants in ClinVar

Classification Summary

Pathogenic116
Likely Pathogenic2
VUS67
Likely Benign9
Benign1
116
Pathogenic
2
Likely Pathogenic
67
VUS
9
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
116
0
116
Likely Pathogenic
0
0
2
0
2
VUS
1
39
27
0
67
Likely Benign
0
7
1
1
9
Benign
0
0
1
0
1
Total1461471195

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

PRXL2B · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found