PRPH

Chr 12ADAR

peripherin

Also known as: NEF4, PRPH1

This gene encodes a cytoskeletal protein found in neurons of the peripheral nervous system. The encoded protein is a type III intermediate filament protein with homology to other cytoskeletal proteins such as desmin, and is a different protein that the peripherin found in photoreceptors. Mutations in this gene have been associated with susceptibility to amyotrophic lateral sclerosis. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

{Amyotrophic lateral sclerosis, susceptibility to}MIM #105400
ADAR
UniProtRetinitis pigmentosa 7
UniProtRetinitis punctata albescens
UniProtMacular dystrophy, vitelliform, 3
UniProtMacular dystrophy, patterned, 1

Clinical highlights

Gene-disease validity (ClinGen)
amyotrophic lateral sclerosis · ADLimitednot for standalone diagnostic reporting
0
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
1.38
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.38LOEUF
pLI 0.000
Z-score 0.17
OE 0.96 (0.681.38)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.56Z-score
OE missense 0.90 (0.811.01)
245 obs / 270.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.96 (0.681.38)
00.351.4
Missense OE?0.90 (0.811.01)
00.61.4
Synonymous OE?0.98
01.21.6
LoF obs/exp: 21 / 21.9Missense obs/exp: 245 / 270.7Syn Z: 0.16

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PRPH · 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 →