MTM1

Chr X

myotubularin 1

Also known as: CNM, CNMX, MTMX, XLMTM

This gene encodes a dual-specificity phosphatase that acts on both phosphotyrosine and phosphoserine. It is required for muscle cell differentiation and mutations in this gene have been identified as being responsible for X-linked myotubular myopathy. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtMyopathy, centronuclear, X-linked

Clinical highlights

Gene-disease validity (ClinGen)
X-linked myotubular myopathy · XLDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype) and the gene is intolerant of it in the population — truncating, frameshift and canonical splice variants carry more prior weight here than missense.Curated gene-level mechanism — a prior for triage, not a per-variant call.
4
Active trials
32
Pubs (1 yr)
P/LP submissions
P/LP missense
0.18
LOEUF· LoF intol.
LOF
Mechanism· G2P
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GeneReview available — MTM1
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

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.18LOEUF
pLI 1.000
Z-score 4.63
OE 0.04 (0.010.18)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
2.39Z-score
OE missense 0.57 (0.490.65)
138 obs / 243.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.04 (0.010.18)
00.351.4
Missense OE?0.57 (0.490.65)
00.61.4
Synonymous OE?0.77
01.21.6
LoF obs/exp: 1 / 26.9Missense obs/exp: 138 / 243.1Syn Z: 1.65

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MTM1 · protein map & ClinVar variants

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

VCEP specificationsCongenital MyopathiesReleased
Panel ↗