PMP2

Chr 8

peripheral myelin protein 2

Also known as: CMT1G, FABP8, M-FABP, MP2, P2

The protein encoded by this gene localizes to myelin sheaths of the peripheral nervous system. The encoded protein can bind both the membrane layers of the sheaths and monomeric lipids, and is thought to provide stability to the sheath. A defect in this gene was shown to be a cause of dominant demyelinating CMT neuropathy. [provided by RefSeq, Jan 2017]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCharcot-Marie-Tooth disease, demyelinating, type 1G

Clinical highlights

Gene-disease validity (ClinGen)
Charcot-Marie-Tooth disease · ADModerateconsider for supplementary testing
0
Active trials
Pubs (1 yr)
P/LP submissions
P/LP missense
1.30
LOEUF
DN
Mechanism· predicted
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GeneReview available — PMP2
Authoritative clinical overview · Recommended first read
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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?
1.30LOEUF
pLI 0.008
Z-score 1.05
OE 0.57 (0.281.30)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.10Z-score
OE missense 0.97 (0.791.18)
67 obs / 69.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.57 (0.281.30)
00.351.4
Missense OE?0.97 (0.791.18)
00.61.4
Synonymous OE?0.86
01.21.6
LoF obs/exp: 4 / 7.0Missense obs/exp: 67 / 69.3Syn Z: 0.55

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PMP2 · 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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