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Updated: May 9, 2026

A Phenotyping Regimen for Genetically Modified Mice Used to Study Genes Implicated in Human Diseases of Aging
09:37

A Phenotyping Regimen for Genetically Modified Mice Used to Study Genes Implicated in Human Diseases of Aging

Published on: July 14, 2016

Progressive HNF1A-MODY pathophysiology revealed by a translational mouse model.

Isaline Louvet1, Ana Acosta-Montalvo1, Chiara Saponaro1

  • 1University of Lille, CHU Lille, Inserm U1190, European Genomic Institute for Diabetes, Institut Pasteur de Lille, Lille, France.

JCI Insight
|May 8, 2026
PubMed
Summary
This summary is machine-generated.

Hepatocyte Nuclear Factor 1-Alpha (HNF1A)-MODY, a genetic diabetes, progresses due to β cell dysfunction. New HNF1A-haploinsufficient mice reveal early insulin resistance and multi-organ issues, offering a better disease model.

Keywords:
AgingBeta cellsDiabetesEndocrinologyMetabolism

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Area of Science:

  • Endocrinology
  • Genetics
  • Metabolic Diseases

Background:

  • Hepatocyte Nuclear Factor 1-Alpha (HNF1A)-Maturity-Onset Diabetes of the Young) (MODY) is the most common monogenic diabetes, characterized by progressive β cell dysfunction.
  • Existing mouse models do not accurately reflect the temporal progression of human HNF1A-MODY, hindering the study of its pathogenesis.

Purpose of the Study:

  • To develop and characterize a novel mouse model that recapitulates human HNF1A haploinsufficiency and the progressive nature of HNF1A-MODY.
  • To investigate the underlying mechanisms of HNF1A-MODY, including β cell function, insulin resistance, and multi-organ involvement.

Main Methods:

  • Generation of mice with heterozygous deletion of the Hnf1a transactivation domain (Hnf1a+/Δe4-10) to mimic human HNF1A haploinsufficiency.
  • Cross-sectional metabolic characterization of Hnf1a+/Δe4-10 mice at various ages.
  • Validation of findings in HNF1A-deficient human islets with HNF1A knockdown.

Main Results:

  • Hnf1a+/Δe4-10 mice exhibited temporal HNF1A-MODY progression, including early insulin resistance, testosterone deficiency, hyperglycemia, and glucose intolerance.
  • Aged mice showed multi-organ dysfunction (liver, kidney) with elevated hepatic gluconeogenesis, impaired renal glucose handling, and hepatic steatosis/fibrosis.
  • Human islet studies confirmed reduced SGLT2, inappropriate glucagon/insulin secretion, and translational relevance.

Conclusions:

  • The Hnf1a+/Δe4-10 mouse model accurately recapitulates human HNF1A-MODY pathophysiology.
  • Early insulin resistance is identified as a key driver of hormonal dysfunction and multi-organ pathophysiology in HNF1A-MODY.
  • HNF1A plays a critical role in maintaining multi-organ homeostasis beyond its traditional role in β cell function.