DES Chromosome 2

Desmin
181 variants 181 Health Risk

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What This Gene Does
This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
Intermediate filaments Type III
Locus Type
gene with protein product
Location
2q35
Ensembl
ENSG00000175084
Associated Conditions (21)
Cardiomyopathy
Desmin-related myofibrillar myopathy
Cardiovascular phenotype
Dilated cardiomyopathy 1I
Neurogenic scapuloperoneal syndrome
Kaeser type
Arrhythmogenic right ventricular cardiomyopathy
DES-related disorder
Left ventricular noncompaction cardiomyopathy
See cases
Primary familial hypertrophic cardiomyopathy
Neuromuscular disease
Primary dilated cardiomyopathy
Primary familial dilated cardiomyopathy
Dilated cardiomyopathy 1S
Congenital diaphragmatic hernia
Myofibrillar myopathy
Bradycardia
Progressive familial heart block
Limb-girdle muscular dystrophy
+1 more conditions
Key Variants
RS1014762661
Conflicting classifications of pathogenicity
Cardiomyopathy, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
Health Risk
RS1057523274
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
Health Risk
RS1064794869
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
Health Risk
RS112224037
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Dilated cardiomyopathy 1I
Health Risk
RS1157722667
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
Health Risk
RS121913002
Conflicting classifications of pathogenicity
Dilated cardiomyopathy 1I, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
Health Risk
RS1292042317
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Dilated cardiomyopathy 1I
Health Risk
RS1339030541
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
Health Risk
RS1400593451
Conflicting classifications of pathogenicity
Arrhythmogenic right ventricular cardiomyopathy, Cardiomyopathy, Desmin-related myofibrillar myopathy
Health Risk
RS140222667
Conflicting classifications of pathogenicity
Neurogenic scapuloperoneal syndrome, Kaeser type, Dilated cardiomyopathy 1I
Health Risk
RS140375681
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Cardiomyopathy, Desmin-related myofibrillar myopathy
Health Risk
RS143154982
Conflicting classifications of pathogenicity
Desmin-related myofibrillar myopathy, Cardiovascular phenotype, DES-related disorder
Health Risk
All Variants (181)
RSID Category Clinical Significance Conditions
RS1014762661 Health Risk Conflicting classifications of pathogenicity Cardiomyopathy, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
RS1057523274 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS1064794869 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS112224037 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Dilated cardiomyopathy 1I
RS1157722667 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS121913002 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
RS1292042317 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Dilated cardiomyopathy 1I
RS1339030541 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS1400593451 Health Risk Conflicting classifications of pathogenicity Arrhythmogenic right ventricular cardiomyopathy, Cardiomyopathy, Desmin-related myofibrillar myopathy
RS140222667 Health Risk Conflicting classifications of pathogenicity Neurogenic scapuloperoneal syndrome, Kaeser type, Dilated cardiomyopathy 1I
RS140375681 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiomyopathy, Desmin-related myofibrillar myopathy
RS143154982 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, DES-related disorder
RS144057476 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1I
RS144261171 Health Risk Conflicting classifications of pathogenicity Left ventricular noncompaction cardiomyopathy, Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1I
RS144901249 Health Risk Conflicting classifications of pathogenicity Cardiomyopathy, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
RS144908941 Health Risk Conflicting classifications of pathogenicity Primary familial hypertrophic cardiomyopathy, Desmin-related myofibrillar myopathy, Cardiovascular phenotype
RS1458306248 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS146755676 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome
RS147327878 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, DES-related disorder
RS148947510 Health Risk Conflicting classifications of pathogenicity Cardiovascular phenotype, Desmin-related myofibrillar myopathy, DES-related disorder
RS150974575 Health Risk Conflicting classifications of pathogenicity Neuromuscular disease, Primary dilated cardiomyopathy, Desmin-related myofibrillar myopathy
RS1553603530 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS1553603571 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Dilated cardiomyopathy 1I
RS1559352868 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome, Kaeser type
RS1954359599 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS1954373441 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS199972656 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome, Kaeser type
RS200545412 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS201458068 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS2125168913 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS2545246557 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS2545255397 Health Risk Conflicting classifications of pathogenicity Cardiomyopathy, Desmin-related myofibrillar myopathy, Cardiomyopathy
RS2545264703 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS267607486 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS267607488 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS267607494 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS267607499 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Neurogenic scapuloperoneal syndrome, Kaeser type
RS34337334 Health Risk Conflicting classifications of pathogenicity Cardiovascular phenotype, Desmin-related myofibrillar myopathy, Cardiomyopathy
RS367961979 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome, Kaeser type
RS370239228 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome
RS371053066 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS372291142 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome, Kaeser type
RS372825868 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Neurogenic scapuloperoneal syndrome
RS375719734 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Neurogenic scapuloperoneal syndrome, Kaeser type
RS397516690 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Desmin-related myofibrillar myopathy
RS397516691 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Cardiovascular phenotype, Desmin-related myofibrillar myopathy
RS397516692 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1I, Neurogenic scapuloperoneal syndrome
RS397516694 Health Risk Conflicting classifications of pathogenicity Desmin-related myofibrillar myopathy, Arrhythmogenic right ventricular cardiomyopathy, Desmin-related myofibrillar myopathy
RS397516695 Health Risk Conflicting classifications of pathogenicity Dilated cardiomyopathy 1S, Desmin-related myofibrillar myopathy, Dilated cardiomyopathy 1S
RS41272699 Health Risk Conflicting classifications of pathogenicity Congenital diaphragmatic hernia, Myofibrillar myopathy, Cardiovascular phenotype
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