Lack of robust satellite cell activation and muscle regeneration during the progression of Pompe disease.

ABSTRACT:

Introduction

Muscle stem cells termed satellite cells are essential for muscle regeneration. A central question in many neuromuscular disorders is why satellite cells are unable to prevent progressive muscle wasting. We have analyzed muscle fiber pathology and the satellite cell response in Pompe disease, a metabolic myopathy caused by acid alpha-glucosidase deficiency and lysosomal glycogen accumulation. Pathology included muscle fiber vacuolization, loss of cross striation, and immune cell infiltration.

Results

The total number of Pax7-positive satellite cells in muscle biopsies from infantile, childhood onset and adult patients (with different ages and disease severities) were indistinguishable from controls, indicating that the satellite cell pool is not exhausted in Pom

SUBMITTER: Schaaf GJ 

PROVIDER: S-EPMC4625612 | biostudies-literature | 2015 Oct

REPOSITORIES: biostudies-literature