Publications by authors named "Anita Muglia"
J Invest Dermatol
July 2024
Article Synopsis
- Recessive dystrophic epidermolysis bullosa (RDEB) is a rare skin disorder caused by mutations in the COL7A1 gene, leading to persistent blistering and fibrosis without a cure.
- The study focuses on the NOTCH signaling pathway, which is implicated in the fibrosis associated with RDEB, and examines the effects of γ-secretase inhibitors, specifically DAPT and PF-03084014 (nirogacestat), on RDEB fibroblasts.
- Results show that inhibiting NOTCH signaling with PF-03084014 reduces fibrotic traits such as contractility and collagen secretion in RDEB fibroblasts, offering potential new therapeutic strategies for managing RDEB-associated fibrosis.
