淋巴管肌瘤病:从分子研究到靶向治疗
Journal of International Pharmaceutical Research（2017）
Abstract
淋巴管肌瘤病(LAM)是一种罕见的肺部疾病,分为散发性LAM(sLAM)和结节性硬化症相关LAM(TSC-LAM)两种,主要见于育龄女性,以肺部囊性病变和血管肌脂瘤或淋巴管肌瘤等腹部肿瘤为主要表现.其发病机制为LAM细胞发生TSC基因突变,导致哺乳动物西罗莫司(雷帕霉素)靶蛋白激酶(mTOR)异常活化,从而致病.mTOR通路在LAM发病中的重要作用推动了mTOR抑制剂(如西罗莫司等)在其治疗中的应用.本文简述了LAM的发病机制,并综述了mTOR抑制剂在LAM治疗中的临床研究进展.
MoreKey words
lymphangioleiomyomatosis,tuberous sclerosis complex,mammalian target of sirolimus(rapamycin),sirolimus,everolimus
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