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Muscle inflammatory pattern in alpha- and gamma-sarcoglycanopathies

Lookup NU author(s): Professor Giorgio TascaORCiD


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AIM: Since the immune system plays a role in the pathogenesis of several muscular dystrophies, we aim to characterize several muscular inflammatory features in α- (LGMD R3) and γ-sarcoglycanopathies (LGMD R5). MATERIALS AND METHODS: We explored the expression of major histocompatibility complex class I molecules (MHCI), and we analyzed the composition of the immune infiltrates in muscle biopsies from 10 patients with LGMD R3 and 8 patients with LGMD R5, comparing the results to Duchenne muscular dystrophy patients (DMD). RESULTS: A consistent involvement of the immune response was observed in sarcoglycanopathies, although it was less evident than in DMD. LGMD R3-R5 and DMD shared an abnormal expression of MHCI, and the composition of the muscular immune cell infiltrate was comparable. CONCLUSION: These findings might serve as a rationale to fine-tune a disease-specific immunomodulatory regimen, particularly relevant in view of the rapid development of gene therapy for sarcoglycanopathies.

Publication metadata

Author(s): Panicucci C, Baratto S, Raffaghello L, Tonin P, D'Amico A, Tasca G, Traverso M, Fiorillo C, Minetti C, Previtali SC, Pegoraro E, Bruno C

Publication type: Article

Publication status: Published

Journal: Clinical neuropathology

Year: 2021

Volume: 40

Issue: 6

Pages: 310-318

Print publication date: 01/11/2021

Acceptance date: 02/04/2018

ISSN (electronic): 0722-5091

Publisher: Dustri-Verlag


DOI: 10.5414/NP301393

PubMed id: 34281632


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