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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SLC12A4
solute carrier family 12 member 4
Chromosome 16 · 16q22.1
NCBI Gene: 6560Ensembl: ENSG00000124067.18HGNC: HGNC:10913UniProt: B4DF30
75PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingATP bindinglysosomal membranepotassium:chloride symporter activityFish-eye diseaseNorum diseaseAbnormality of the cardiovascular systemLCAT deficiency
✦AI Summary

SLC12A4 encodes potassium-chloride cotransporter 1 (KCC1), a ubiquitously expressed electroneutral transporter essential for cellular fluid and ion homeostasis 1. KCC1 mediates K+-Cl- cotransport across plasma membranes and lysosomal membranes, functioning in cell volume regulation and maintaining potassium and chloride ion balance 1. The SLC12A4 promoter lacks a TATA box but contains an initiator element and downstream promoter element regulated by AP-2 and Sp1 transcription factors 2. In erythrocytes, KCC1 contributes significantly to K-Cl cotransport and has been proposed as a modifier gene in sickle cell disease, where elevated K-Cl cotransport occurs 2. KCC1 participates in a chloride-sensing signaling pathway involving WNK1-OSR1-SPAK kinases that controls phagocyte responses during apoptotic cell clearance; loss of SLC12A4 inhibits corpse uptake and anti-inflammatory responses 3. The first disease-associated KCC1 variant (E1065K) reduces cotransporter functional activation under hypotonic conditions, suggesting a role in osmotic regulation 4. SLC12A4 is co-expressed with SLC12A2 in multiple tissues and serves as a therapeutic target; inhibition of both transporters reduces inflammation and angiogenesis in diabetic retinopathy 5. Altered SLC12A4 expression associates with papillary thyroid carcinoma recurrence 6.

Sources cited
1
SLC12A4 encodes a K+-Cl- cotransporter family member involved in cell volume regulation and tissue-specific roles
PMID: 12739168
2
Human KCC1 promoter structure contains InR and DPE elements regulated by AP-2 and Sp1; KCC1 contributes to K-Cl cotransport in erythrocytes and sickle cell disease
PMID: 14976052
3
SLC12A4 participates in chloride-sensing pathway controlling phagocyte efferocytosis and anti-inflammatory responses
PMID: 31792382
4
Disease-associated KCC1 variant E1065K reduces functional activation under hypotonic conditions
PMID: 41414942
5
SLC12A4 inhibition reduces inflammation and angiogenesis in diabetic retinopathy models
PMID: 41789623
6
SLC12A4 expression associates with papillary thyroid carcinoma recurrence prediction
PMID: 34590520
Disease Associationsⓘ20
Fish-eye diseaseOpen Targets
0.50Moderate
Norum diseaseOpen Targets
0.47Moderate
Abnormality of the cardiovascular systemOpen Targets
0.41Moderate
LCAT deficiencyOpen Targets
0.33Weak
neurodegenerative diseaseOpen Targets
0.30Weak
Alzheimer diseaseOpen Targets
0.29Weak
lysosomal storage diseaseOpen Targets
0.29Weak
multiple sclerosisOpen Targets
0.29Weak
Parkinson diseaseOpen Targets
0.29Weak
neuroinflammatory disorderOpen Targets
0.21Weak
Blackfan-Diamond anemiaOpen Targets
0.09Suggestive
inosine triphosphatase deficiencyOpen Targets
0.07Suggestive
autosomal dominant sideroblastic anemiaOpen Targets
0.07Suggestive
Constitutional sideroblastic anemiaOpen Targets
0.07Suggestive
alpha thalassemia-intellectual disability syndrome type 1Open Targets
0.06Suggestive
Alpha-thalassemia - intellectual disability syndrome linked to chromosome 16Open Targets
0.06Suggestive
delta-beta-thalassemiaOpen Targets
0.06Suggestive
X-linked sideroblastic anemia 1Open Targets
0.06Suggestive
Heinz body anemiaOpen Targets
0.06Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PSKH1Protein interaction83%WNK3Protein interaction83%WNK1Protein interaction80%WNK4Protein interaction79%SLC12A6Shared pathway78%SLC12A7Shared pathway63%
Tissue Expression6 tissues
Heart
100%
Lung
95%
Ovary
59%
Liver
42%
Bone Marrow
22%
Brain
19%
Gene Interaction Network
Click a node to explore
SLC12A4PSKH1WNK3WNK1WNK4SLC12A6SLC12A7
PROTEIN STRUCTURE
Preparing viewer…
PDB6KKR · 2.90 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.83LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.69 [0.58–0.83]
RankingsWhere SLC12A4 stands among ~20K protein-coding genes
  • #6,365of 20,598
    Most Researched75
  • #7,079of 17,882
    Most Constrained (LOEUF)0.83
Genes detectedSLC12A4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Human potassium chloride cotransporter 1 (SLC12A4) promoter is regulated by AP-2 and contains a functional downstream promoter element.
PMID: 14976052
Blood · 2004
1.00
2
Bumetanide‑blocked SLC12A2 exerts a protective effect in experimental diabetic retinopathy.
PMID: 41789623
Int J Mol Med · 2026
0.90
3
Interpreting an apoptotic corpse as anti-inflammatory involves a chloride sensing pathway.
PMID: 31792382
Nat Cell Biol · 2019
0.80
4
The erythroid K-Cl cotransport inhibitor [(dihydroindenyl)oxy]acetic acid blocks erythroid Ca
PMID: 35848620
Am J Physiol Cell Physiol · 2022
0.70
5
Exploring the Cell Biological and Functional Effects of the First Disease Associated KCC1 Genetic Variant.
PMID: 41414942
J Cell Physiol · 2025
0.60