SCNN1A Chromosome 12

Sodium channel epithelial 1 subunit alpha
60 variants 60 Health Risk

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What This Gene Does
Nonvoltage-gated, amiloride-sensitive, sodium channels control fluid and electrolyte transport across epithelia in many organs. These channels are heteromeric complexes consisting of 3 subunits: alpha, beta, and gamma. This gene encodes the alpha subunit, and mutations in this gene have been associated with pseudohypoaldosteronism type 1 (PHA1), a rare salt wasting disease resulting from target organ unresponsiveness to mineralocorticoids. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Apr 2009]
Gene Info
Gene Group
Sodium channels epithelial
Locus Type
gene with protein product
Location
12p13.31
Ensembl
ENSG00000111319
Associated Conditions (17)
Bronchiectasis with or without elevated sweat chloride 2
Pseudohypoaldosteronism
type IB1
autosomal recessive
Liddle syndrome 3
Inborn genetic diseases
Malignant tumor of urinary bladder
Gastric cancer
Thyroid cancer
nonmedullary
1
Cervical cancer
SCNN1A-related disorder
Idiopathic bronchiectasis
Incidental Discovery
Neurodevelopmental delay
Renal tubulopathies
Key Variants
RS1366336758
Conflicting classifications of pathogenicity
Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
Health Risk
RS137852634
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS138995556
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS139335335
Conflicting classifications of pathogenicity
Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
Health Risk
RS140681881
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS142409152
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS144275086
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS146177203
Conflicting classifications of pathogenicity
Bronchiectasis with or without elevated sweat chloride 2, Liddle syndrome 3, Pseudohypoaldosteronism
Health Risk
RS148749888
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS148959452
Conflicting classifications of pathogenicity
Pseudohypoaldosteronism, type IB1, autosomal recessive
Health Risk
RS149484264
Conflicting classifications of pathogenicity
Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
Health Risk
RS199526819
Conflicting classifications of pathogenicity
Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
Health Risk
All Variants (60)
RSID Category Clinical Significance Conditions
RS1366336758 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS137852634 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS138995556 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS139335335 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS140681881 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS142409152 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS144275086 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS146177203 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Liddle syndrome 3, Pseudohypoaldosteronism
RS148749888 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS148959452 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS149484264 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS199526819 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS200557019 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Pseudohypoaldosteronism, type IB1
RS200770604 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS201235216 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS201873521 Health Risk Conflicting classifications of pathogenicity Liddle syndrome 3, Pseudohypoaldosteronism, type IB1
RS367681929 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Pseudohypoaldosteronism, type IB1
RS369382063 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS370406973 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS375712066 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS376456435 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS3764873 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS539360032 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS55981728 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS569195112 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS61758858 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS62619209 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS62620999 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS72657550 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS72657556 Health Risk Conflicting classifications of pathogenicity
RS751903373 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS755287192 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS759933475 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Pseudohypoaldosteronism, type IB1
RS761663786 Health Risk Conflicting classifications of pathogenicity
RS771949339 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, SCNN1A-related disorder, Liddle syndrome 3
RS772498945 Health Risk Conflicting classifications of pathogenicity Bronchiectasis with or without elevated sweat chloride 2, Bronchiectasis with or without elevated sweat chloride 2
RS775290860 Health Risk Conflicting classifications of pathogenicity Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1165464622 Health Risk Likely pathogenic
RS1369791519 Health Risk Likely pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS137852635 Health Risk Likely pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1555112332 Health Risk Likely pathogenic Idiopathic bronchiectasis, Idiopathic bronchiectasis
RS1565488675 Health Risk Likely pathogenic Bronchiectasis with or without elevated sweat chloride 2, Liddle syndrome 3, Pseudohypoaldosteronism
RS2497879030 Health Risk Likely pathogenic SCNN1A-related disorder, SCNN1A-related disorder
RS2497885818 Health Risk Likely pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1565478701 Health Risk Pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1592074026 Health Risk Pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1948331954 Health Risk Pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS1948588956 Health Risk Pathogenic
RS2136865918 Health Risk Pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
RS2497930442 Health Risk Pathogenic Pseudohypoaldosteronism, type IB1, autosomal recessive
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