P2X7 Receptor Antagonist Reduces Fibrosis and Inflammation in a Mouse Model of Alpha-Sarcoglycan Muscular Dystrophy

• Main Authors: Lizzia Raffaghello (Author), Elisa Principi (Author), Serena Baratto (Author), Chiara Panicucci (Author), Sara Pintus (Author), Francesca Antonini (Author), Genny Del Zotto (Author), Andrea Benzi (Author), Santina Bruzzone (Author), Paolo Scudieri (Author), Carlo Minetti (Author), Elisabetta Gazzerro (Author), Claudio Bruno (Author)
• Format: Book
• Published: MDPI AG, 2022-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Limb-girdle muscular dystrophy R3, a rare genetic disorder affecting the limb proximal muscles, is caused by mutations in the α-sarcoglycan gene (Sgca) and aggravated by an immune-mediated damage, finely modulated by the extracellular (e)ATP/purinoceptors axis. Currently, no specific drugs are avail...