Nuclear aggregates in oculopharyngeal muscular dystrophy

被引：0
|
作者：
Boulinguiez, Alexis [1 ]
Roth, Fany [1 ]
Mouigni, Hadidja Rose [1 ]
Butler-Browne, Gillian [1 ]
Mouly, Vincent [1 ]
Trollet, Capucine [1 ]
机构：
[1] Sorbonne Univ Inserm, Inst Myol, Ctr Rech Myol, Paris, France
来源：
M S-MEDECINE SCIENCES | 2023年 / 38卷
关键词：
PROTEIN AGGREGATION; MOUSE MODEL; INCLUSIONS; CHAPERONES;
D O I：
10.1051/medsci/2022175
中图分类号：
R-3 [医学研究方法]; R3 [基础医学];
学科分类号：
1001 ;
摘要：
Oculopharyngeal muscular dystrophy (OPMD) is one of the diseases related to pathological expansions of trinucleotides. Its pathogenesis remains unclear although the presence of aggregates within the nuclei of the muscle fiber seems to play an important role. The basic research studies presented here help understand their composition and their deleterious role. These elements may result in new therapeutic avenues.
引用
收藏
页码：13 / 16
页数：4
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