致死性癫痫性 Lafora 病的淀粉样变性抵抗自噬性糖原分解代谢。
Amylopectinosis of the fatal epilepsy Lafora disease resists autophagic glycogen catabolism.
机构信息
Division of Neurology, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, 75390, USA.
Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah Hebrew University Medical Center, Jerusalem, 9112001, Israel.
出版信息
EMBO Mol Med. 2024 May;16(5):1047-1050. doi: 10.1038/s44321-024-00063-9. Epub 2024 Apr 2.
Abstract
In this Correspondence, B. Minassian and colleagues report that GHF201, an autophagy activator shown to diminish abnormal glycogen aggregates in a mouse model of Adult Polyglucosan Body Disease, fails to reduce such accumulations in a mouse model of Lafora disease. [Image: see text]
摘要
在这封通信中，B. Minassian 及其同事报告称，GHF201 是一种自噬激活剂，在成人多聚糖体病的小鼠模型中可减少异常糖原聚集体，但其在 Lafora 病的小鼠模型中并不能减少此类聚集体的积累。[图片：见正文]
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