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GeneE
25 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
SCNN1A
sodium channel epithelial 1 subunit alpha
Chromosome 12 Β· 12p13.31
NCBI Gene: 6337Ensembl: ENSG00000111319.14HGNC: HGNC:10599UniProt: P37088
219PubMed Papers
23Diseases
5Drugs
39Pathogenic Variants
FUNCTIONAL ROLE
Ion ChannelTransporter
RESEARCH IMPACT
Trending
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
apical plasma membranecytoplasmextracellular exosomeciliary membranepseudohypoaldosteronism, type IB1, autosomal recessivebronchiectasis with or without elevated sweat chloride 2Generalized pseudohypoaldosteronism type 1hypertension
✦AI Summary

SCNN1A encodes the alpha subunit of the epithelial sodium channel (ENaC), a critical component for sodium ion transport across epithelial membranes 1. The primary function involves sodium ion homeostasis and regulation of blood pressure through renal sodium reabsorption 2. Mechanistically, SCNN1A mutations can cause either loss-of-function, leading to pseudohypoaldosteronism type 1 with salt wasting and hyperkalemia 1, or gain-of-function, resulting in Liddle syndrome characterized by hypertension and hypokalemia 23. The p.Phe226Cys mutation causes an 83% reduction in ENaC activity through decreased protein expression and reduced channel open probability 1. Disease relevance extends beyond electrolyte disorders, as SCNN1A is overexpressed in pancreatic cancer where it promotes cell proliferation, migration, and invasion 4. It also serves as a prognostic biomarker in triple-negative breast cancer, with high expression associated with poor neoadjuvant chemotherapy response 5. Additionally, SCNN1A polymorphisms are linked to neonatal respiratory distress syndrome in term infants 6. Clinical significance includes its potential as a therapeutic target and biomarker across multiple diseases, from inherited electrolyte disorders to cancer metastasis 7.

Sources cited
1
SCNN1A mutations cause Liddle syndrome with hypertension and hypokalemia
PMID: 39236685
2
SCNN1A mutations cause pseudohypoaldosteronism type 1 and the p.Phe226Cys mutation reduces ENaC activity by 83%
PMID: 37134141
3
Novel SCNN1A mutations cause Liddle syndrome with autosomal dominant inheritance
PMID: 36336351
4
SCNN1A is overexpressed in pancreatic cancer and promotes cellular growth and metastasis
PMID: 35714697
5
High SCNN1A expression in triple-negative breast cancer is associated with poor prognosis and neoadjuvant therapy response
PMID: 40287704
6
SCNN1A polymorphisms are associated with neonatal respiratory distress syndrome in term infants
PMID: 26611714
7
SCNN1A serves as a non-invasive biomarker for ovarian cancer metastasis
PMID: 40787466
Disease Associationsβ“˜23
pseudohypoaldosteronism, type IB1, autosomal recessiveOpen Targets
0.78Strong
bronchiectasis with or without elevated sweat chloride 2Open Targets
0.75Strong
Generalized pseudohypoaldosteronism type 1Open Targets
0.72Strong
hypertensionOpen Targets
0.61Moderate
congestive heart failureOpen Targets
0.58Moderate
cardiac arrhythmiaOpen Targets
0.56Moderate
bronchiectasisOpen Targets
0.55Moderate
Liddle syndrome 3Open Targets
0.54Moderate
pseudohypoaldosteronism type 1Open Targets
0.53Moderate
edemaOpen Targets
0.53Moderate
cardiovascular diseaseOpen Targets
0.51Moderate
preeclampsiaOpen Targets
0.46Moderate
nephrotic syndromeOpen Targets
0.46Moderate
cirrhosis of liverOpen Targets
0.46Moderate
Liddle syndrome 2Open Targets
0.46Moderate
idiopathic bronchiectasisOpen Targets
0.44Moderate
Liddle syndromeOpen Targets
0.37Weak
hyperaldosteronismOpen Targets
0.37Weak
Brugada syndromeOpen Targets
0.37Weak
ciliopathyOpen Targets
0.37Weak
Bronchiectasis with or without elevated sweat chloride 2UniProt
Liddle syndrome 3UniProt
Pseudohypoaldosteronism 1B1, autosomal recessiveUniProt
Pathogenic Variants39
NM_001038.6(SCNN1A):c.574del (p.Arg192fs)Pathogenic
Pseudohypoaldosteronism|Bronchiectasis with or without elevated sweat chloride 2;Pseudohypoaldosteronism, type IB1, autosomal recessive;Liddle syndrome 3|not provided|SCNN1A-related disorder
β˜…β˜…β˜†β˜†2026β†’ Residue 192
NM_001038.6(SCNN1A):c.1579TTC[1] (p.Phe528del)Pathogenic
Neurodevelopmental delay|Renal tubulopathies
β˜…β˜…β˜†β˜†2025β†’ Residue 528
NM_001038.6(SCNN1A):c.1449del (p.Tyr484fs)Pathogenic
Pseudohypoaldosteronism, type IB1, autosomal recessive|not provided|Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2;Liddle syndrome 3|Incidental Discovery
β˜…β˜…β˜†β˜†2025β†’ Residue 484
NM_001038.6(SCNN1A):c.1360+2T>GPathogenic
Pseudohypoaldosteronism, type IB1, autosomal recessive
β˜…β˜…β˜†β˜†2025
NM_001038.6(SCNN1A):c.1474C>T (p.Arg492Ter)Pathogenic
Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2;Liddle syndrome 3|Pseudohypoaldosteronism, type IB1, autosomal recessive
β˜…β˜…β˜†β˜†2024β†’ Residue 492
NM_001038.6(SCNN1A):c.505_506del (p.Thr169fs)Pathogenic
Liddle syndrome 3;Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 169
NM_001038.6(SCNN1A):c.875+1G>APathogenic
not provided|Pseudohypoaldosteronism, type IB1, autosomal recessive|Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2;Liddle syndrome 3
β˜…β˜…β˜†β˜†2024
NM_001038.6(SCNN1A):c.166C>T (p.Arg56Ter)Pathogenic
not provided
β˜…β˜…β˜†β˜†2023β†’ Residue 56
NM_001038.6(SCNN1A):c.1439+1G>APathogenic
Pseudohypoaldosteronism, type IB1, autosomal recessive|Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2;Liddle syndrome 3
β˜…β˜…β˜†β˜†2022
NM_001038.6(SCNN1A):c.285del (p.Phe95fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2026β†’ Residue 95
NM_001038.6(SCNN1A):c.1332dup (p.Cys445fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 445
NM_001038.6(SCNN1A):c.1374_1375del (p.Tyr458_Lys459delinsTer)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 458
NM_001038.6(SCNN1A):c.19del (p.Glu7fs)Likely pathogenic
Bronchiectasis with or without elevated sweat chloride 2
β˜…β˜†β˜†β˜†2025β†’ Residue 7
NM_001038.6(SCNN1A):c.1520C>A (p.Ser507Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 507
NM_001038.6(SCNN1A):c.885_886del (p.His296fs)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 296
NM_001038.6(SCNN1A):c.685-1G>ALikely pathogenic
Liddle syndrome 3;Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2
β˜…β˜†β˜†β˜†2024
NM_001038.6(SCNN1A):c.979+1G>ALikely pathogenic
Liddle syndrome 3;Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2
β˜…β˜†β˜†β˜†2024
NM_001038.6(SCNN1A):c.203_204del (p.Ile68fs)Pathogenic
Pseudohypoaldosteronism, type IB1, autosomal recessive
β˜…β˜†β˜†β˜†2024β†’ Residue 68
NM_001038.6(SCNN1A):c.1678G>A (p.Gly560Ser)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 560
NM_001038.6(SCNN1A):c.979G>T (p.Gly327Cys)Likely pathogenic
Liddle syndrome 3;Pseudohypoaldosteronism, type IB1, autosomal recessive;Bronchiectasis with or without elevated sweat chloride 2
β˜…β˜†β˜†β˜†2024β†’ Residue 327
View on ClinVar β†—
Drug Targets5
AMILORIDEApproved
Amiloride-sensitive sodium channel, ENaC blocker
cardiovascular disease
AMILORIDE HYDROCHLORIDEApproved
Amiloride-sensitive sodium channel, ENaC blocker
COFIRASERSENPhase I
Amiloride-sensitive sodium channel, ENaC mRNA antisense inhibitor
cystic fibrosis
IDREVLORIDEPhase II
Amiloride-sensitive sodium channel, ENaC blocker
cystic fibrosis
TRIAMTERENEApproved
Amiloride-sensitive sodium channel, ENaC blocker
hypertension
Related Genes
NEDD4Protein interaction100%SLC12A3Protein interaction97%SCN5AProtein interaction95%SCNN1BProtein interaction93%SCNN1GProtein interaction93%SCN3BProtein interaction88%
Tissue Expression6 tissues
Lung
100%
Brain
86%
Liver
16%
Ovary
5%
Bone Marrow
4%
Heart
1%
Gene Interaction Network
Click a node to explore
SCNN1ANEDD4SLC12A3SCN5ASCNN1BSCNN1GSCN3B
PROTEIN STRUCTURE
Preparing viewer…
PDB6WTH Β· 3.06 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.10LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.89 [0.72–1.10]
RankingsWhere SCNN1A stands among ~20K protein-coding genes
  • #1,888of 20,598
    Most Researched219 Β· top 10%
  • #462of 1,025
    FDA-Approved Drug Targets3
  • #1,560of 5,498
    Most Pathogenic Variants39
  • #11,299of 17,882
    Most Constrained (LOEUF)1.10
Genes detectedSCNN1A
Sources retrieved25 papers
Response timeβ€”
πŸ“„ Sources
25β–Ό
1
Liddle Syndrome with a SCNN1A Mutation: A Case Report and Literature Review.
PMID: 39236685
Kidney Blood Press Res Β· 2024
1.00
2
Integrative single-cell and exosomal multi-omics uncovers SCNN1A and EFNA1 as non-invasive biomarkers and drivers of ovarian cancer metastasis.
PMID: 40787466
Front Immunol Β· 2025
0.90
3
A mild and transient form of autosomal recessive pseudohypoaldosteronism type 1 caused by a novel mutation in the
PMID: 37134141
Am J Physiol Endocrinol Metab Β· 2023
0.80
4
Clinical and genetic characteristics of the patients with hypertension and hypokalemia carrying a novel
PMID: 36336351
Scand J Clin Lab Invest Β· 2022
0.70
5
Hormonal regulation of non-cystic fibrosis transmembrane conductance regulator ion channels in the endocervix.
PMID: 36907435
F S Sci Β· 2023
0.68