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Exploring the tumors of multiple endocrine neoplasia type 1 in mouse models for basic and preclinical studies
1National Institutes of Health, NIDDK, Metabolic Diseases Branch, Bldg 10, Room 8C-101, Bethesda, MD 20892, USA, Tel.: +1 301 402 7834.
Abstract:
Most patients (70-90%) with the multiple endocrine neoplasia type 1 (MEN1) syndrome possess germline heterozygous mutations in MEN1 that predisposes to tumors of multiple endocrine and nonendocrine tissues. Some endocrine tumors of the kinds seen in MEN1 that occur sporadically in the general population also possess somatic mutations in MEN1. Interestingly, the endocrine tumors of MEN1 are recapitulated in mouse models of Men1 loss that serve as a valuable resource to understand the pathophysiology and molecular basis of tumorigenesis. Exploring these endocrine tumors in mouse models using in vivo, ex vivo and in vitro methods can help to follow the process of tumorigenesis, and can be useful for preclinical testing of therapeutics and understanding their mechanisms of action.
Insights
Multiple Endocrine Neoplasia type 1 (MEN1) is linked to MEN1 gene mutations, leading to various tumors. Mouse models of MEN1 loss are crucial for studying tumor development and testing new therapies.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Multiple Endocrine Neoplasia type 1 (MEN1) syndrome is characterized by germline mutations in the MEN1 gene.
- These mutations predispose patients to tumors in endocrine and nonendocrine tissues.
- Sporadic endocrine tumors also exhibit somatic MEN1 mutations.
Purpose of the Study:
- To investigate the role of MEN1 gene mutations in tumorigenesis.
- To utilize mouse models for understanding MEN1-associated tumor development.
- To explore the potential of mouse models for preclinical therapeutic testing.
Main Methods:
- Utilizing mouse models with Men1 loss.
- Employing in vivo, ex vivo, and in vitro experimental approaches.
- Analyzing the pathophysiology and molecular basis of tumorigenesis.
Main Results:
- Mouse models of Men1 loss recapitulate endocrine tumors seen in human MEN1.
- These models provide a valuable resource for studying tumor development.
- The models facilitate the understanding of tumorigenesis processes.
Conclusions:
- Mouse models are essential for understanding MEN1 pathophysiology.
- These models aid in the preclinical evaluation of therapeutics for MEN1-related tumors.
- Further exploration of these models can advance the development of targeted treatments.
