H Lawrence Remmel, Sandra S Hammer, Laurence A Neff, Olivier M Dorchies, Leonardo Scapozza, Dirk Fischer, Steven C Quay
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引用次数: 0
摘要
杜兴氏肌肉萎缩症(DMD)是一种遗传性 X 连锁疾病,具有进展性、衰弱性和最终致命性。目前的治疗方法还不能治愈该病,但包括延缓疾病进展的缓和疗法,以及有望治愈该病的基因疗法。新疗法还有很大的发展空间,雌激素受体调节剂他莫昔芬的基础研究表明,他莫昔芬的代谢产物内托昔芬有可能带来独特的治疗效果。在此,我们将结合 DMD 的总体治疗情况,介绍这种新型 DMD 治疗方法的潜力。
A Hypothesized Therapeutic Role of (Z)-Endoxifen in Duchenne Muscular Dystrophy (DMD).
Duchenne Muscular Dystrophy (DMD) is an inherited, X-linked disorder that is progressive, debilitating, and ultimately fatal. The current therapeutic landscape offers no cures, but does include palliative treatments that delay disease progression, and there is progress on genetic therapies that have the promise to be curative. There is much room for new therapies, and foundational work with the estrogen receptor modulator tamoxifen suggests the potential of a unique spectrum of therapeutic benefit from endoxifen, a metabolite of tamoxifen. Here we describe the potential for this new DMD therapy in the context of the overall DMD therapeutic landscape.