PRB1

Chr 12

proline rich protein BstNI subfamily 1

Also known as: PM, PMF, PMS, PRB1L, PRB1M

The PRB1 protein is predicted to be located in the extracellular region, but its specific function and clinical significance remain unclear. No well-established diseases have been definitively linked to PRB1 mutations in the medical literature. The gene shows very low constraint against loss-of-function variants, suggesting it may be tolerant to disruption.

Summary from RefSeq
Research Assistant →
0
Active trials
11
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.92
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryPRB1
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
1.92LOEUF
pLI 0.000
Z-score -1.31
OE 1.48 (0.941.92)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-4.03Z-score
OE missense 2.12 (1.802.00)
216 obs / 101.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.48 (0.941.92)
00.351.4
Missense OE2.12 (1.802.00)
00.61.4
Synonymous OE1.98
01.21.6
LoF obs/exp: 13 / 8.8Missense obs/exp: 216 / 101.7Syn Z: -4.65
DN
0.91top 5%
GOF
0.92top 5%
LOF
0.3161th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PRB1 · 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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Clinical Literature
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