RHPN1

Chr 8

rhophilin Rho GTPase binding protein 1

Also known as: ODF5, RHOPHILIN, RHPN

RHPN1 encodes a protein that serves as a target for Rho signaling and interacts with cytoskeletal components to regulate stress fiber assembly and focal adhesion formation. Mutations cause autosomal recessive nephronophthisis with retinal dystrophy, typically presenting in childhood with progressive kidney disease and vision loss. The gene shows very low constraint against loss-of-function variants, consistent with a recessive inheritance pattern.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.11
Clinical SummaryRHPN1
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
1.11LOEUF
pLI 0.000
Z-score 1.08
OE 0.78 (0.561.11)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.41Z-score
OE missense 0.94 (0.871.03)
388 obs / 411.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.561.11)
00.351.4
Missense OE0.94 (0.871.03)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 22 / 28.2Missense obs/exp: 388 / 411.2Syn Z: -0.96

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RHPN1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

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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