Genetic and Molecular Determinants of Lymphatic Malformations: Potential Targets for Therapy.
Genetic and Molecular Determinants of Lymphatic Malformations: Potential Targets for Therapy.
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DOI：
10.3390/jdb10010011
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发表时间：
2022-02-08
影响因子：
2.7
通讯作者：
Farmer DL
中科院分区：
文献类型：
--
作者：
Lee SY;Loll EG;Hassan AS;Cheng M;Wang A;Farmer DL
淋巴管畸形是一种液体填充的先天性淋巴管缺陷，发生率为16,000至16,000。有各种类型，它们往往与其他先天性异常和血管畸形一起存在。近年来，在了解这些畸形方面取得了很大进展。本文综述了淋巴管生成的分子和胚胎学前体。基因突变和失调牵连淋巴管畸形的发病机制进行了讨论。最后，我们触及当前和发展中的疗法，特别关注靶向生物治疗。
Lymphatic malformations are fluid-filled congenital defects of lymphatic channels occurring in 1 in 6000 to 16,000 patients. There are various types, and they often exist in conjunction with other congenital anomalies and vascular malformations. Great strides have been made in understanding these malformations in recent years. This review summarize known molecular and embryological precursors for lymphangiogenesis. Gene mutations and dysregulations implicated in pathogenesis of lymphatic malformations are discussed. Finally, we touch on current and developing therapies with special attention on targeted biotherapeutics.