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dilated cardiomyopathy 1Z (CMD1Z) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

dilated cardiomyopathy 1Z

MONDO:0012745

Also known as:CMD1ZTNNC1 familial isolated dilated cardiomyopathycardiomyopathy, dilated, type 1Z

Overview

Any familial isolated dilated cardiomyopathy in which the cause of the disease is a mutation in the TNNC1 gene.

GeneticsAutosomal dominant · TNNC1 (chr 3)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Congestive heart failure and Enlarged and weakened heart (dilated cardiomyopathy); and sometimes findings: Sudden cardiac death.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	3	Congestive heart failure, Sudden cardiac death, Enlarged and weakened heart (dilated cardiomyopathy)

Explore clinical features

Genetic Landscape

TNNC1 function has not been fully characterized.

Dilated cardiomyopathy 1Z is caused by mutations in the TNNC1 gene on chromosome 3.

Diagnosis

Genetic testing for TNNC1 is available. Testing is considered confirmatory for diagnosis.

Prognosis

Phenotype severity distribution: 2 very common features.

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 7:17 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:611879

GARD

Genetic and Rare Diseases Info Center

GARD:15531

Questions for Your Care Team

→Should I pursue genetic testing for TNNC1, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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dilated cardiomyopathy 1Z

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Prognosis

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team