PRX

Chr 19

periaxin

Also known as: CMT4F

This gene encodes a protein involved in peripheral nerve myelin upkeep. The encoded protein contains 2 PDZ domains which were named after PSD95 (post synaptic density protein), DlgA (Drosophila disc large tumor suppressor), and ZO1 (a mammalian tight junction protein). Two alternatively spliced transcript variants have been described for this gene which encode different protein isoforms and which are targeted differently in the Schwann cell. Mutations in this gene cause Charcot-Marie-Tooth neuoropathy, type 4F and Dejerine-Sottas neuropathy. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDejerine-Sottas syndrome
UniProtCharcot-Marie-Tooth disease, demyelinating, type 4F

Clinical highlights

Gene-disease validity (ClinGen)
Charcot-Marie-Tooth disease type 4 · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
213
Pubs (1 yr)
P/LP submissions
P/LP missense
0.98
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — PRX
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.98LOEUF
pLI 0.000
Z-score 1.63
OE 0.71 (0.530.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.13Z-score
OE missense 1.01 (0.961.07)
862 obs / 851.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.71 (0.530.98)
00.351.4
Missense OE?1.01 (0.961.07)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 27 / 37.9Missense obs/exp: 862 / 851.2Syn Z: -0.01

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PRX · protein map & ClinVar variants

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