Animal models of MEN1

Area of Science:

• Oncology
• Endocrinology
• Genetics

Background:

• Animal models are vital for understanding cancer biology, gene function, and preclinical therapy testing.
• The multiple endocrine neoplasia type 1 (MEN1) syndrome is studied using various animal models with mutations in the human MEN1 gene orthologues.

Purpose of the Study:

• To review existing and potential animal models for MEN1 and MEN1-like syndromes.
• To discuss the contribution of these models to understanding Menin function in tumorigenesis and disease biology.
• To highlight the utility of models in preclinical therapy response studies for neuroendocrine tumors (NETs).

Main Methods:

• Review of established and emerging animal models for MEN1 syndrome.
• Analysis of genetic mutations (e.g., Men1, Cdkn1b) in Drosophila, mice, and rats.
• Discussion of preclinical therapy response studies using MEN1 models.

Main Results:

• Menin's function in tumorigenesis is conserved across species from flies to mammals.
• Mouse models closely mimic human MEN1 tumors and hormonal changes, though frequencies vary.
• MENX rats (Cdkn1b mutation) exhibit MEN1-like features and are used for NET therapy testing.

Conclusions:

• Animal models, including mice and rats, are indispensable tools for MEN1 research.
• These models have significantly advanced the understanding of MEN1 biology and identified potential therapies for NETs.
• Future models like patient-derived xenografts may further enhance preclinical studies for MEN1-associated tumors.