MTMR2

Chr 11

myotubularin related protein 2

Also known as: CMT4B, CMT4B1

This gene is a member of the myotubularin family of phosphoinositide lipid phosphatases. The encoded protein possesses phosphatase activity towards phosphatidylinositol-3-phosphate and phosphatidylinositol-3,5-bisphosphate. Mutations in this gene are a cause of Charcot-Marie-Tooth disease type 4B, an autosomal recessive demyelinating neuropathy. Alternatively spliced transcript variants encoding multiple isoforms have been found for this gene. [provided by RefSeq, Aug 2011]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCharcot-Marie-Tooth disease, demyelinating, type 4B1

Clinical highlights

Gene-disease validity (ClinGen)
demyelinating hereditary motor and sensory neuropathy · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
0.65
LOEUF
DN
Mechanism· predicted
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GeneReview available — MTMR2
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?
0.65LOEUF
pLI 0.000
Z-score 3.31
OE 0.44 (0.300.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.22Z-score
OE missense 0.81 (0.730.90)
272 obs / 334.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.44 (0.300.65)
00.351.4
Missense OE?0.81 (0.730.90)
00.61.4
Synonymous OE?0.76
01.21.6
LoF obs/exp: 18 / 40.8Missense obs/exp: 272 / 334.7Syn Z: 2.02

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MTMR2 · 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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