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MEN-1和MEN-2的表型及其发生的分子机制多发性内分泌肿瘤(multiple endocrineneoplasia,MEN)是一类较少见的与家族遗传有关的内分泌肿瘤。其主要特征是同一个体的2个或2个以上内分泌器官发生肿瘤,且同一个器官常存在2个或以上的肿瘤。以往根据肿瘤谱的不同,将MEN分为MEN-1和MEN-2,其中MEN-2又可分为MEN-2A和MEN-2B。MEN-1的病变主要累及甲状旁腺、垂体和胰腺;MEN-2主要累及甲状腺、肾上腺和甲状旁腺,其中,MEN-2B还可伴有黏膜神经瘤和马凡综合征样表现。MEN-1系常染色体显性遗传疾病,其致病的分子遗传学机制是抑癌基因MEN1失活,该基因定位于染色体11q13,其编
Phenotypes of MEN-1 and MEN-2 and Their Molecular Mechanisms Multiple endocrine neoplasia (MEN) is a rare family-related endocrine neoplasm. Its main feature is that two or more endocrine organs of the same individual have tumors, and two or more tumors often exist in the same organ. In the past, according to the tumor spectrum, MEN was divided into MEN-1 and MEN-2, and MEN-2 was divided into MEN-2A and MEN-2B. MEN-1 mainly affects the parathyroid, pituitary and pancreas lesions. MEN-2 mainly affects the thyroid, adrenal and parathyroid glands. MEN-2B may be associated with mucosal neuroma and Marfan syndrome. MEN-1 Department of autosomal dominant genetic disease, its molecular pathogenesis of molecular pathogenesis of tumor suppressor gene MEN1 inactivation, the gene located on chromosome 11q13, its series