Werner syndrome
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MEN-1 (Werner syndrome)
![The Werner syndrome protein confers resistance to the DNA lesions N3-methyladenine and O^6-methylguanine: implications for WRN function [An article from: DNA Repair]](https://lh3.googleusercontent.com/blogger_img_proxy/AEn0k_uACxnY589AqD51NnxVgTpf4SilVniTGW7NTZYZNFgEBLZIJn2hELS-dK0X8QgZbrq582kDZQ5W4_1SmhLjHC9Mmr7lbr90qAO3TJPo-9GH-Z3nu_cxSXLwhBt8YTGh4FWC3wxUALeGhpf0DWCSWuQJaecGjz0zsCxVlk7W-Fzbrklkpe1VjRk_vHlh_nTKIPt58X8LwqvQBUhmdxVEqnCjsYTFEqafpQ_cd0vf6hI=s0-d)
3 Ps:
- parathyroid
- pancreas
- pituitary
- the most common – and usually the first - manifestation of MEN-1
- can cause hypercalcemia (and related things, like kidney stones)
- pancreatic carcinoma
- big cause of morbidity/mortality
- often metastatic by the time of diagnosis
- can secrete stuff (gastrin, insulin) that causes symptoms
- pituitary adenoma
- prolactinoma most common
other stuff
- duodenal gastrinoma
- carcinoid
- adenomas of thyroid and adrenal cortex
- lipomas
Genetic stuff
MEN1 gene
- mutation in MEN1 gene
- MEN1 gene encodes menin (a nuclear protein that probably regulates transcription and the cell cycle)
- MEN1 is a classic tumor-suppressor gene (need to inactivate both alleles of the gene to get tumors)
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