MYOZ2

Chr 4AD

myozenin 2

Also known as: C4orf5, CMH16, CS-1, FATZ-2

The protein encoded by this gene belongs to a family of sarcomeric proteins that bind to calcineurin, a phosphatase involved in calcium-dependent signal transduction in diverse cell types. These family members tether calcineurin to alpha-actinin at the z-line of the sarcomere of cardiac and skeletal muscle cells, and thus they are important for calcineurin signaling. Mutations in this gene cause cardiomyopathy familial hypertrophic type 16, a hereditary heart disorder. [provided by RefSeq, Aug 2011]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Cardiomyopathy, hypertrophic, 16MIM #613838
AD
UniProtCardiomyopathy, familial hypertrophic, 16

Clinical highlights

Gene-disease validity (ClinGen)
hypertrophic cardiomyopathy · ADDisputedevidence questions this relationship
0
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
1.07
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — MYOZ2
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.07LOEUF
pLI 0.002
Z-score 1.41
OE 0.54 (0.301.07)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.54Z-score
OE missense 0.87 (0.751.01)
125 obs / 143.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.54 (0.301.07)
00.351.4
Missense OE?0.87 (0.751.01)
00.61.4
Synonymous OE?1.01
01.21.6
LoF obs/exp: 6 / 11.1Missense obs/exp: 125 / 143.2Syn Z: -0.07

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MYOZ2 · 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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