Muscle inflammatory pattern in alpha- and gamma-sarcoglycanopathies

被引：6

作者：

Panicucci, Chiara ^{[1
]}

Baratto, Serena ^{[1
]}

Raffaghello, Lizzia ^{[1
]}

Tonin, Paola ^{[2
]}

D'Amico, Adele ^{[3
]}

Tasca, Giorgio ^{[4
]}

Traverso, Monica ^{[5
,6
]}

Fiorillo, Chiara ^{[5
,6
]}

Minetti, Carlo ^{[5
,6
]}

Previtali, Stefano Carlo ^{[7
,8
]}

Pegoraro, Elena ^{[9
]}

Bruno, Claudio ^{[1
]}

机构：

[1] IRCCS Ist Giannina Gaslini, Ctr Translat & Expt Myol, Via Gerolamo Gaslini 5, I-16147 Genoa, Italy

[2] Univ Verona, Neurol Clin, Verona, Italy

[3] IRCCS Bambino Gesu Childrens Hosp, Unit Neuromuscular & Neurodegenerat Disorders, Rome, Italy

[4] Fdn Policlin Univ A Gemelli IRCCS, Unita Operat Complessa Neurol, Rome, Italy

[5] IRCCS Ist Giannina Gaslini, Pediat Neurol & Neuromuscular Disorders Unit, Genoa, Italy

[6] Univ Genoa, Genoa, Italy

[7] IRCSS San Raffaele Sci Inst, Neuromuscular Repair Unit, Inspe, Milan, Italy

[8] IRCSS San Raffaele Sci Inst, Div Neurosci, Milan, Italy

[9] Univ Padua, ERN Neuromuscular Ctr, Dept Neurosci, Padua, Italy

来源：

CLINICAL NEUROPATHOLOGY | 2021年 / 40卷 / 06期

关键词：

sarcoglycanopathy; DAMPS; Duchenne muscular dystrophy; limb-girdle muscular dystrophies; inflammation; MAJOR HISTOCOMPATIBILITY COMPLEX; MUSCULAR-DYSTROPHY; CLASS-I; EXPRESSION; PATHOLOGY; CELLS; MICE; LIFE;

D O I：

10.5414/NP301393

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Aim: Since the immune system plays a role in the pathogenesis of several muscular dystrophies, we aim to characterize several muscular inflammatory features in alpha- (LGMD R3) and gamma-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.

引用

页码：310 / 318

页数：9

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