maturity-onset diabetes of the young type 13
Findings
No curated finding names maturity-onset diabetes of the young type 13 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Any maturity-onset diabetes of the young in which the cause of the disease is a mutation in the KCNJ11 gene.
Definition from the Mondo Disease Ontology (MONDO:0014589), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Infantile onset · Childhood onset · Intermediate young adult onset · Late young adult onset
HPO, annotations 2026-09-02
Features
7 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Maturity-onset diabetes of the youngHPOHP:0004904
- 4 of 4 reported patients
- Elevated hemoglobin A1cHPOHP:0040217
- 3 of 4 reported patients
- Anti-glutamic acid decarboxylase antibody positivityHPOHP:0025329
- 1 of 4 reported patients
- Decreased circulating C-peptide concentrationHPOHP:0030795
- 1 of 4 reported patients
- Diabetes mellitusHPOHP:0000819
- 1 of 4 reported patients
- HyperglycemiaHPOHP:0003074
- 1 of 4 reported patients
- Abnormality of body mass index
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- KCNJ11HGNC:6257
- Strong · Genomics England PanelApp · Autosomal dominant · 2020
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2022
- Strong · PanelApp Australia · Autosomal dominant · 2025
Where it sits
- A kind of
Other names
3 names
Resolves to: maturity-onset diabetes of the young type 13
- Also called
- KCNJ11 maturity-onset diabetes of the young (disease)maturity-onset diabetes of the young (disease) caused by mutation in KCNJ11MODY13