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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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KCNH6
potassium voltage-gated channel subfamily H member 6
Chromosome 17 · 17q23.3
NCBI Gene: 81033Ensembl: ENSG00000173826.16HGNC: HGNC:18862UniProt: B4DKC0
20PubMed Papers
20Diseases
7Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Ion ChannelTransporter
CLINICAL
FDA Approved Target
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
regulation of ventricular cardiac muscle cell membrane repolarizationpotassium ion transmembrane transportmembrane repolarization during cardiac muscle cell action potentialregulation of heart rate by cardiac conductionmultiple sclerosisneurodegenerative diseaseMyasthenia gravisLambert-Eaton myasthenic syndrome
✦AI Summary

KCNH6 encodes a pore-forming subunit of voltage-gated inwardly rectifying potassium channels characterized by distinctive gating kinetics: rapid inactivation during depolarization with slow deactivation during repolarization, resulting in large inward currents 1. Primary functions include potassium ion transmembrane transport and regulation of cardiac and pancreatic cell membrane potential 12. Mechanistically, KCNH6 regulates pancreatic β-cell insulin secretion by controlling membrane depolarization and intracellular calcium homeostasis 13. The channel protects β-cells from endoplasmic reticulum (ER) stress-induced apoptosis; KCNH6 knockout increases ER stress, intracellular calcium, and β-cell apoptosis, while overexpression attenuates ER stress responses 2. Additionally, m6A RNA modification regulates KCNH6 expression in a YTHDF1-dependent manner, controlling fibroblast-to-myofibroblast transition in pulmonary fibrosis 4. Disease relevance includes: KCNH6 mutations causing hypoinsulinemia and hyperglycemia in humans and mice 32; involvement in pulmonary fibrosis pathogenesis 4; and potential role as a glioblastoma prognostic biomarker 5. Clinical significance: Berberine and cisapride inhibit KCNH6 currents to stimulate glucose-dependent insulin secretion, with berberine demonstrating efficacy in human clinical trials without causing hypoglycemia 13. KCNH6-targeting represents a promising therapeutic strategy for diabetes treatment.

Sources cited
1
KCNH6 regulates insulin secretion through membrane depolarization control; berberine targets KCNH6 to stimulate insulin secretion in mouse models and human clinical trials
PMID: 34556670
2
KCNH6 protects pancreatic β-cells from ER stress-induced apoptosis; KCNH6 knockout impairs glucose tolerance with increased ER stress and apoptosis
PMID: 32918525
3
Cisapride inhibits KCNH6 currents to promote insulin secretion and reduce blood glucose; effect is KCNH6-dependent in β-cells
PMID: 36325440
4
KCNH6 expression is regulated by m6A RNA modification in a YTHDF1-dependent manner and involved in pulmonary fibrosis pathogenesis
PMID: 34111558
5
KCNH6 is identified as a glioblastoma prognostic biomarker and potential target for molecular therapy
PMID: 39612412
Disease Associationsⓘ20
multiple sclerosisOpen Targets
0.59Moderate
neurodegenerative diseaseOpen Targets
0.58Moderate
Myasthenia gravisOpen Targets
0.56Moderate
Lambert-Eaton myasthenic syndromeOpen Targets
0.55Moderate
Muscle weaknessOpen Targets
0.46Moderate
congenital myasthenic syndromeOpen Targets
0.43Moderate
Congenital myasthenic syndromesOpen Targets
0.43Moderate
neoplasmOpen Targets
0.37Weak
autoimmune diseaseOpen Targets
0.37Weak
autoimmune disorder of the nervous systemOpen Targets
0.37Weak
cancerOpen Targets
0.37Weak
cardiac arrhythmiaOpen Targets
0.37Weak
immune system diseaseOpen Targets
0.37Weak
Increased muscle fatiguabilityOpen Targets
0.37Weak
nervous system diseaseOpen Targets
0.37Weak
nervous system neoplasmOpen Targets
0.37Weak
neuromuscular diseaseOpen Targets
0.37Weak
neuromuscular junction diseaseOpen Targets
0.37Weak
paraneoplastic neurologic syndromeOpen Targets
0.37Weak
small cell carcinomaOpen Targets
0.37Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets7
AMIFAMPRIDINEApproved
Voltage-gated potassium channel blocker
Myasthenia gravis
AMIFAMPRIDINE PHOSPHATEApproved
Voltage-gated potassium channel blocker
Lambert-Eaton myasthenic syndrome
DALFAMPRIDINEApproved
Voltage-gated potassium channel blocker
multiple sclerosis
GUANIDINEPhase III
Voltage-gated potassium channel blocker
neuroendocrine neoplasm
GUANIDINE HYDROCHLORIDEApproved
Voltage-gated potassium channel blocker
Myasthenia gravis
NERISPIRDINEPhase II
Voltage-gated potassium channel blocker
multiple sclerosis
TEDISAMILApproved
Voltage-gated potassium channel blocker
cardiac arrhythmia
Related Genes
EBPProtein interaction90%KCNA4Protein interaction88%SIGMAR1Protein interaction88%ERGProtein interaction73%KCNH2Protein interaction65%KCNU1Shared pathway50%
Tissue Expression6 tissues
Brain
100%
Lung
2%
Bone Marrow
1%
Liver
0%
Ovary
0%
Heart
0%
Gene Interaction Network
Click a node to explore
KCNH6EBPKCNA4SIGMAR1ERGKCNH2KCNU1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9H252
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.14LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.95 [0.79–1.14]
RankingsWhere KCNH6 stands among ~20K protein-coding genes
  • #14,176of 20,598
    Most Researched20
  • #336of 1,025
    FDA-Approved Drug Targets5
  • #11,836of 17,882
    Most Constrained (LOEUF)1.14
Genes detectedKCNH6
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Berberine is an insulin secretagogue targeting the KCNH6 potassium channel.
PMID: 34556670
Nat Commun · 2021
1.00
2
m
PMID: 34111558
Mol Ther · 2021
0.90
3
Cisapride induced hypoglycemia
PMID: 36325440
Front Endocrinol (Lausanne) · 2022
0.80
4
Identification and validation of ion channels-related mRNA prognostic signature for glioblastomas.
PMID: 39612412
Medicine (Baltimore) · 2024
0.70
5
Short QT syndrome.
PMID: 15890322
Cardiovasc Res · 2005
0.60