KCNJ11 encodes potassium inwardly rectifying channel subfamily J member 11 (390 aa). Inward rectifier potassium channel that forms the pore of ATP-sensitive potassium channels (KATP), regulating potassium permeability as a function of cytoplasmic ATP and ADP concentrations in many different cells. Highest expression in Muscle Skeletal (87.1 TPM) and Brain Cerebellum (37.4 TPM).

Hyperinsulinemic hypoglycemia, familial, 2 is caused by mutations in the KCNJ11 gene on chromosome 11.

The KCNJ11 protein participates in KCNJ11 tetramer:ABCC8 mutants, KCNJ11 tetramer:ABCC8:Mg2+:ADP tetramer, and KCNJ11:ATP tetramer:ABCC8 tetramer pathways.

KCNJ11 is classified as a druggable target (Druggable Genome and Ion Channel categories) with score 1.2.

Genetic testing for KCNJ11 is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for hyperinsulinemic hypoglycemia, familial, 2 has been reported in the published literature.

Phenotype severity distribution: 2 always present features.

1 clinical trial registered. Interventions under study include other interventions. Research is primarily sponsored by academic and government institutions.

88 publications have been identified in PubMed for hyperinsulinemic hypoglycemia, familial, 2. Research spans Case Report / Case Series (35%), Review / Meta-Analysis (22%), and Basic Science / Preclinical (17%).

Recent Publications

• Wong T (2026). [PMID: 41982675](https://pubmed.ncbi.nlm.nih.gov/41982675/). *Front Pharmacol*. [Review / Meta-Analysis]

• Zhang QT (2026). [PMID: 41582760](https://pubmed.ncbi.nlm.nih.gov/41582760/). *Zhongguo Dang Dai Er Ke Za Zhi*. [Review / Meta-Analysis]

• González-Llorens N (2026). [PMID: 41201695](https://pubmed.ncbi.nlm.nih.gov/41201695/). *Paediatr Drugs*. [Review / Meta-Analysis]

• Widmer A (2026). [PMID: 40705962](https://pubmed.ncbi.nlm.nih.gov/40705962/). *J Clin Endocrinol Metab*. [Case Report / Case Series]

• Rosenfeld E (2026). [PMID: 41980002](https://pubmed.ncbi.nlm.nih.gov/41980002/). *Horm Res Paediatr*. [Review / Meta-Analysis]

• Scala R (2026). [PMID: 40272924](https://pubmed.ncbi.nlm.nih.gov/40272924/). *Diabetes*. [Case Report / Case Series]

• States LJ (2026). [PMID: 41539914](https://pubmed.ncbi.nlm.nih.gov/41539914/). *PET Clin*. [Review / Meta-Analysis]

• Sanders VR (2026). [PMID: 41795819](https://pubmed.ncbi.nlm.nih.gov/41795819/). *Horm Res Paediatr*. [Case Report / Case Series]

• Rossi A (2026). [PMID: 42105432](https://pubmed.ncbi.nlm.nih.gov/42105432/). *Mol Genet Metab*. [Review / Meta-Analysis]

• Kantzavelou A (2026). [PMID: 41978437](https://pubmed.ncbi.nlm.nih.gov/41978437/). *J Clin Res Pediatr Endocrinol*. [Case Report / Case Series]