DSG1 Chromosome 18

Desmoglein 1
46 variants 46 Health Risk

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What This Gene Does
This gene encodes a member of the desmoglein protein subfamily. Desmogleins, along with desmocollins, are cadherin-like transmembrane glycoproteins that are major components of the desmosome. Desmosomes are cell-cell junctions that help resist shearing forces and are found in high concentrations in cells subject to mechanical stress. This gene is found in a cluster with other desmoglein family members on chromosome 18. The encoded protein has been identified as a target of auto-antibodies in the autoimmune skin blistering disease pemphigus foliaceus. Disruption of this gene has also been associated with the skin diseases palmoplantar keratoderma and erythroderma. [provided by RefSeq, Feb 2015]
Gene Info
Gene Group
Desmosomal cadherins
Locus Type
gene with protein product
Location
18q12.1
Ensembl
ENSG00000134760
Associated Conditions (12)
Inborn genetic diseases
Palmoplantar keratoderma i
striate
focal
or diffuse
DSG1-related disorder
Hereditary palmoplantar keratoderma
Bicuspid aortic valve
Abnormality of the skin
Severe dermatitis-multiple allergies-metabolic wasting syndrome
Diffuse palmoplantar hyperkeratosis
Epidermal disease
Key Variants
All Variants (46)
RSID Category Clinical Significance Conditions
RS112716438 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS144825489 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS146862520 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS147377474 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS200143796 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS201665237 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS202198738 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS2071779127 Health Risk Conflicting classifications of pathogenicity Palmoplantar keratoderma i, striate, focal
RS376868157 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS556017565 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS61730307 Health Risk Conflicting classifications of pathogenicity DSG1-related disorder, Inborn genetic diseases, DSG1-related disorder
RS946952164 Health Risk Conflicting classifications of pathogenicity
RS989159105 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS1057518788 Health Risk Likely pathogenic Hereditary palmoplantar keratoderma, Bicuspid aortic valve, Hereditary palmoplantar keratoderma
RS1203908934 Health Risk Likely pathogenic DSG1-related disorder, DSG1-related disorder
RS2144093610 Health Risk Likely pathogenic Abnormality of the skin, Abnormality of the skin
RS2144107587 Health Risk Likely pathogenic Severe dermatitis-multiple allergies-metabolic wasting syndrome, Severe dermatitis-multiple allergies-metabolic wasting syndrome
RS2144110924 Health Risk Likely pathogenic Palmoplantar keratoderma i, striate, focal
RS2510846042 Health Risk Likely pathogenic
RS761241711 Health Risk Likely pathogenic Hereditary palmoplantar keratoderma, Diffuse palmoplantar hyperkeratosis, Hereditary palmoplantar keratoderma
RS1268442199 Health Risk Pathogenic
RS1436840056 Health Risk Pathogenic
RS145081747 Health Risk Pathogenic Severe dermatitis-multiple allergies-metabolic wasting syndrome, Palmoplantar keratoderma i, striate
RS1568039793 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS1598699465 Health Risk Pathogenic
RS2144077500 Health Risk Pathogenic
RS2144091452 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS2144098983 Health Risk Pathogenic
RS2510845796 Health Risk Pathogenic
RS2511042832 Health Risk Pathogenic
RS2511043841 Health Risk Pathogenic
RS2511043894 Health Risk Pathogenic DSG1-related disorder, DSG1-related disorder
RS2511043976 Health Risk Pathogenic
RS2511045840 Health Risk Pathogenic
RS2511047532 Health Risk Pathogenic
RS397515639 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS397515640 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS397515641 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS398122400 Health Risk Pathogenic Severe dermatitis-multiple allergies-metabolic wasting syndrome, Severe dermatitis-multiple allergies-metabolic wasting syndrome
RS398122949 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS398122950 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS398122951 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS398122952 Health Risk Pathogenic Severe dermatitis-multiple allergies-metabolic wasting syndrome, Severe dermatitis-multiple allergies-metabolic wasting syndrome
RS568609861 Health Risk Pathogenic Palmoplantar keratoderma i, striate, focal
RS1182196436 Health Risk Pathogenic/Likely pathogenic Palmoplantar keratoderma i, striate, focal
RS1463828394 Health Risk Pathogenic/Likely pathogenic Severe dermatitis-multiple allergies-metabolic wasting syndrome, Severe dermatitis-multiple allergies-metabolic wasting syndrome
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