CLRN1

Chr 3AR

clarin 1

Also known as: RP61, USH3, USH3A

This gene encodes a protein that contains a cytosolic N-terminus, multiple helical transmembrane domains, and an endoplasmic reticulum membrane retention signal, TKGH, in the C-terminus. The encoded protein may be important in development and homeostasis of the inner ear and retina. Mutations within this gene have been associated with Usher syndrome type IIIa. Multiple transcript variants encoding distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 1.802 OMIM phenotypes
Clinical SummaryCLRN1
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Gene-Disease Validity (ClinGen)
Usher syndrome type 3 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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?
1.80LOEUF
pLI 0.000
Z-score -0.49
OE 1.17 (0.731.80)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.59Z-score
OE missense 1.15 (1.001.31)
150 obs / 130.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.17 (0.731.80)
00.351.4
Missense OE?1.15 (1.001.31)
00.61.4
Synonymous OE?0.96
01.21.6
LoF obs/exp: 11 / 9.4Missense obs/exp: 150 / 130.9Syn Z: 0.24

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CLRN1 · 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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