maturity-onset diabetes of the young type 1
MONDO:0007452Mondo
Findings
No curated finding names maturity-onset diabetes of the young type 1 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Monogenic diabetes caused by inactivating mutation(s) in the gene HNF4A, encoding hepatocyte nuclear factor 4-alpha.
Definition from the Mondo Disease Ontology (MONDO:0007452), read 2026-09-29. CC BY 4.0.
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.
- HNF4AHGNC:5024
- Definitive · Ambry Genetics · Autosomal dominant · 2018
- Definitive · G2P · Autosomal dominant · 2025
- Strong · Genomics England PanelApp · Autosomal dominant · 2021
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
- Strong · PanelApp Australia · Autosomal dominant · 2025
Where it sits
- A kind of
Other names
6 names
Resolves to: maturity-onset diabetes of the young type 1
- Also called
- hepatocyte nuclear Factor 4-Alpha associated monogenic diabetesHNF4A-associated monogenic diabetesmaturity onset diabetes of the Young, type 1mild juvenile diabetes mellitusMODY, type IMODY1