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GeneE
26 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
SLC12A2
solute carrier family 12 member 2
Chromosome 5 Β· 5q23.3
NCBI Gene: 6558Ensembl: ENSG00000064651.15HGNC: HGNC:10911UniProt: B7ZM24
174PubMed Papers
23Diseases
0Drugs
30Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Trending
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
cytoplasmic vesicle membraneT cell chemotaxistransepithelial chloride transportammonium channel activityDelpire-McNeill syndromehearing loss, autosomal dominant 78Kilquist syndromeVaricose veins
✦AI Summary

SLC12A2 encodes the Na-K-2Cl cotransporter-1 (NKCC1), a cation-chloride cotransporter mediating electroneutral transport of chloride, potassium, and sodium ions across cell membranes 1. It plays a vital role in regulating ionic balance and cell volume 1. NKCC1 is particularly important in the thick ascending limb of the renal loop of Henle, where mutations cause Bartter syndrome characterized by renal salt wasting, hypokalemic metabolic alkalosis, and hypercalciuria 2. Loss-of-function SLC12A2 mutations are associated with neurodevelopmental deficits, deafness (autosomal dominant deafness 78), and variable fluid secretion abnormalities across epithelia 3. The gene appears to be haploinsufficient, with single allelic deleterious variants causing disease in some patients 3. Beyond inherited disorders, SLC12A2 dysfunction has broader clinical relevance: ammonium uptake through SLC12A2 promotes drug resistance in multiple myeloma by stabilizing NEK2 protein 4, and altered SLC12A2 expression is associated with increased breast cancer risk 5. Dysregulated SLC12A2 has also been implicated in osteoporosis pathogenesis 6. Current therapeutic approaches include loop diuretics like furosemide that downregulate SLC12A2 expression to mitigate pathological effects 4.

Sources cited
1
SLC12A2 mediates electroneutral transport of chloride, potassium, and sodium ions and regulates ionic balance and cell volume
PMID: 16669787
2
SLC12A2 mutations cause Bartter syndrome with renal salt wasting, hypokalemic metabolic alkalosis, and hypercalciuria
PMID: 12920401
3
SLC12A2 mutations cause neurodevelopmental deficits, deafness, and variable fluid secretion abnormalities; gene is haploinsufficient
PMID: 37399495
4
Ammonium uptake through SLC12A2 promotes drug resistance in multiple myeloma; furosemide downregulates SLC12A2 to improve treatment response
PMID: 38113887
5
Decreased SLC12A2 expression is associated with increased breast cancer risk, particularly estrogen receptor positive tumors
PMID: 38396187
6
SLC12A2 is a key gene in endoplasmic reticulum stress and mitochondrial dysfunction related to osteoporosis pathogenesis
PMID: 39953608
Disease Associationsβ“˜23
Delpire-McNeill syndromeOpen Targets
0.73Strong
hearing loss, autosomal dominant 78Open Targets
0.68Moderate
Kilquist syndromeOpen Targets
0.66Moderate
Varicose veinsOpen Targets
0.52Moderate
Abnormality of the skeletal systemOpen Targets
0.50Moderate
Intellectual disabilityOpen Targets
0.49Moderate
hearing lossOpen Targets
0.49Moderate
cellulitisOpen Targets
0.47Moderate
sensorineural hearing lossOpen Targets
0.46Moderate
lymphatic system diseaseOpen Targets
0.43Moderate
edemaOpen Targets
0.42Moderate
genetic disorderOpen Targets
0.42Moderate
deafnessOpen Targets
0.40Weak
pelvic organ prolapseOpen Targets
0.38Weak
ConstipationOpen Targets
0.37Weak
ectodermal dysplasia syndromeOpen Targets
0.37Weak
Familial intestinal malrotation - facial anomaliesOpen Targets
0.37Weak
Intestinal malrotationOpen Targets
0.37Weak
multiple congenital anomalies/dysmorphic syndrome-intellectual disabilityOpen Targets
0.37Weak
volvulus of midgutOpen Targets
0.37Weak
Deafness, autosomal dominant, 78UniProt
Delpire-McNeill syndromeUniProt
Kilquist syndromeUniProt
Pathogenic Variants30
NM_001046.3(SLC12A2):c.3312del (p.Phe1104fs)Pathogenic
Inborn genetic diseases|not provided|SLC12A2-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 1104
NM_001046.3(SLC12A2):c.2962C>T (p.Pro988Ser)Pathogenic
Sensorineural hearing loss disorder|not provided
β˜…β˜…β˜†β˜†2022β†’ Residue 988
NM_001046.3(SLC12A2):c.3100+2T>CLikely pathogenic
SLC12A2-related disorder
β˜…β˜†β˜†β˜†2025
NM_001046.3(SLC12A2):c.352dup (p.Ala118fs)Likely pathogenic
Delpire-McNeill syndrome
β˜…β˜†β˜†β˜†2025β†’ Residue 118
NM_001046.3(SLC12A2):c.2977+1G>ALikely pathogenic
Hearing loss, autosomal dominant 78
β˜…β˜†β˜†β˜†2025
NM_001046.3(SLC12A2):c.541G>T (p.Glu181Ter)Likely pathogenic
Delpire-McNeill syndrome
β˜…β˜†β˜†β˜†2025β†’ Residue 181
NM_001046.3(SLC12A2):c.1881+2T>CLikely pathogenic
SLC12A2-related disorder
β˜…β˜†β˜†β˜†2025
NM_001046.3(SLC12A2):c.2959C>T (p.Gln987Ter)Likely pathogenic
Hearing loss, autosomal dominant 78
β˜…β˜†β˜†β˜†2025β†’ Residue 987
NM_001046.3(SLC12A2):c.2160_2163dup (p.Leu722fs)Likely pathogenic
Delpire-McNeill syndrome
β˜…β˜†β˜†β˜†2024β†’ Residue 722
NM_001046.3(SLC12A2):c.2935G>A (p.Glu979Lys)Pathogenic
Hearing loss|Hearing loss, autosomal dominant 78|not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 979
NM_001046.3(SLC12A2):c.959C>A (p.Ser320Ter)Likely pathogenic
Kilquist syndrome
β˜…β˜†β˜†β˜†2024β†’ Residue 320
NM_001046.3(SLC12A2):c.2617-2A>GPathogenic
Kilquist syndrome
β˜…β˜†β˜†β˜†2024
NM_001046.3(SLC12A2):c.2935G>T (p.Glu979Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2023β†’ Residue 979
NM_001046.3(SLC12A2):c.1279G>T (p.Gly427Ter)Likely pathogenic
SLC12A2-related disorder
β˜…β˜†β˜†β˜†2023β†’ Residue 427
NM_001046.3(SLC12A2):c.3147G>A (p.Trp1049Ter)Likely pathogenic
Hearing loss, autosomal dominant 78;Delpire-McNeill syndrome;Kilquist syndrome
β˜…β˜†β˜†β˜†2023β†’ Residue 1049
NM_001046.3(SLC12A2):c.2476-1G>CLikely pathogenic
Delpire-McNeill syndrome
β˜…β˜†β˜†β˜†2022
NM_001046.3(SLC12A2):c.2371_2372insTAAATTAGGCCACT (p.Cys791delinsLeuAsnTer)Pathogenic
not provided
β˜…β˜†β˜†β˜†2022β†’ Residue 791
NM_001046.3(SLC12A2):c.1059T>G (p.Tyr353Ter)Likely pathogenic
See cases
β˜…β˜†β˜†β˜†2021β†’ Residue 353
NM_001046.3(SLC12A2):c.1228C>T (p.Arg410Ter)Pathogenic
SLC12A2-related disorder
β˜†β˜†β˜†β˜†2023β†’ Residue 410
NM_001046.3(SLC12A2):c.1431del (p.Phe477fs)Pathogenic
Kilquist syndrome
β˜†β˜†β˜†β˜†2020β†’ Residue 477
View on ClinVar β†—
Related Genes
PRKACAProtein interaction98%PRKACGProtein interaction98%PRKACBProtein interaction98%ADCY3Protein interaction95%ADCY9Protein interaction95%KCNQ1Protein interaction95%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
41%
Heart
38%
Ovary
23%
Lung
17%
Liver
11%
Gene Interaction Network
Click a node to explore
SLC12A2PRKACAPRKACGPRKACBADCY3ADCY9KCNQ1
PROTEIN STRUCTURE
Preparing viewer…
PDB9C0H Β· 2.50 Γ… Β· EM
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.72LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.50 [0.35–0.72]
RankingsWhere SLC12A2 stands among ~20K protein-coding genes
  • #2,534of 20,598
    Most Researched174 Β· top quartile
  • #1,794of 5,498
    Most Pathogenic Variants30
  • #5,566of 17,882
    Most Constrained (LOEUF)0.72
Genes detectedSLC12A2
Sources retrieved26 papers
Response timeβ€”
πŸ“„ Sources
26β–Ό
1
Secretory diarrhoea: mechanisms and emerging therapies.
PMID: 26122478
Nat Rev Gastroenterol Hepatol Β· 2015
1.00
2
Expanding the genetic heterogeneity of intellectual disability.
PMID: 28940097
Hum Genet Β· 2017
0.90
3
NKCC1 in human diseases: is the
PMID: 37399495
Am J Physiol Cell Physiol Β· 2023
0.80
4
Targeting gut microbial nitrogen recycling and cellular uptake of ammonium to improve bortezomib resistance in multiple myeloma.
PMID: 38113887
Cell Metab Β· 2024
0.70
5
Chloride deregulation and GABA depolarization in MTOR-related malformations of cortical development.
PMID: 39106285
Brain Β· 2025
0.68