Satellite cells fail to contribute to muscle repair but are functional in Pompe disease (glycogenosis type II)

Satellite cells fail to contribute to muscle repair but are functional in Pompe disease (glycogenosis type II)

Abstract Pompe disease, which is due to acid alpha-glucosidase deficiency, is characterized by skeletal muscle dysfunction attributed to the accumulation of glycogen-filled lysosomes and autophagic buildup. Despite the extensive tissue damages, a failure of satellite cell (SC) activation and lack of...

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Bibliographic Details
Main Authors: Lydie Lagalice, Julien Pichon, Eliot Gougeon, Salwa Soussi, Johan Deniaud, Mireille Ledevin, Virginie Maurier, Isabelle Leroux, Sylvie Durand, Carine Ciron, Francesca Franzoso, Laurence Dubreil, Thibaut Larcher, Karl Rouger, Marie-Anne Colle
Format: Article
Language:English
Published: BMC 2018-10-01
Series:Acta Neuropathologica Communications
Subjects:
Pompe disease
GAA KO 6neo/6neo mice
Skeletal muscle
Satellite cell
Muscle regeneration
Lysosome
Online Access:http://link.springer.com/article/10.1186/s40478-018-0609-y

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http://link.springer.com/article/10.1186/s40478-018-0609-y

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