Features include: Maturity-onset diabetes of the young, Polycystic ovaries, and Hepatocellular adenoma.

HNF1A encodes HNF1 homeobox A (631 aa). Transcriptional activator that regulates the tissue specific expression of multiple genes, especially in pancreatic islet cells and in liver. Binds to the inverted palindrome 5'-GTTAATNATTAAC-3'. Highest expression in Liver (8.7 TPM) and Small Intestine Terminal Ileum (6.3 TPM).

Hepatic adenomas, familial is associated with mutations in the HNF1A gene on chromosome 12.

The HNF1A protein participates in HNF1A-dependent synthesis of FOXA3, HNF1A-dependent synthesis of HNF4A, and HNF1A-dependent synthesis of GLUT2 protein pathways.

HNF1A is classified as a druggable target (Clinically Actionable and Transcription Factor categories) with score 26.1.

Genetic testing for HNF1A is available. Testing is considered confirmatory for diagnosis.

No clinical trials have been registered for hepatic adenomas, familial.

6 publications have been identified in PubMed for hepatic adenomas, familial. Research spans Review / Meta-Analysis (33%), Basic Science / Preclinical (33%), and Case Report / Case Series (17%).

Recent Publications

• Choi JH (2025). [PMID: 39442859](https://pubmed.ncbi.nlm.nih.gov/39442859/). *Journal of Yeungnam medical science*. [Review / Meta-Analysis]

• Bergignat S (2025). [PMID: 40078582](https://pubmed.ncbi.nlm.nih.gov/40078582/). *Frontiers in endocrinology*. [Epidemiology / Natural History]

• Drda E (2025). [PMID: 41146957](https://pubmed.ncbi.nlm.nih.gov/41146957/). *Journal of surgical case reports*. [Case Report / Case Series]

• Reguram R (2024). [PMID: 39470338](https://pubmed.ncbi.nlm.nih.gov/39470338/). *Hepatology communications*. [Review / Meta-Analysis]

• Faini AC (2024). [PMID: 39408812](https://pubmed.ncbi.nlm.nih.gov/39408812/). *International journal of molecular sciences*. [Basic Science / Preclinical]