Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies.

Szczegóły
Abstrakt

Tytuł:: Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies.
Autorzy:: Farini A; Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Villa C; Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Tripodi L; Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Legato M; Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Torrente Y; Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.
Źródło:: Frontiers in immunology [Front Immunol] 2021 Jul 14; Vol. 12, pp. 666879. Date of Electronic Publication: 2021 Jul 14 (Print Publication: 2021).
Typ publikacji:: Journal Article; Research Support, Non-U.S. Gov't; Review
Język:: English
Imprint Name(s):: Original Publication: [Lausanne : Frontiers Research Foundation]
MeSH Terms:: Autoimmunity*
Immunoglobulins/*immunology
Muscular Dystrophies/*immunology
Myositis/*immunology
Autoantibodies/immunology ; Humans ; Immunoglobulins/metabolism ; Muscle Proteins/metabolism ; Muscular Dystrophies/metabolism ; Muscular Dystrophies/pathology ; Myositis/metabolism ; Myositis/pathology
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Contributed Indexing:: Keywords: autoantibodies; autoimmunity; immunoglobulins; inflammatory myopathies; muscle inflammation; muscular dystrophies
Substance Nomenclature:: 0 (Autoantibodies)
0 (Immunoglobulins)
0 (Muscle Proteins)
Entry Date(s):: Date Created: 20210802 Date Completed: 20211025 Latest Revision: 20240402
Update Code:: 20240402
PubMed Central ID:: PMC8316973
DOI:: 10.3389/fimmu.2021.666879
PMID:: 34335568
: Czasopismo naukowe

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Muscular dystrophies and inflammatory myopathies are heterogeneous muscular disorders characterized by progressive muscle weakness and mass loss. Despite the high variability of etiology, inflammation and involvement of both innate and adaptive immune response are shared features. The best understood immune mechanisms involved in these pathologies include complement cascade activation, auto-antibodies directed against muscular proteins or de-novo expressed antigens in myofibers, MHC-I overexpression in myofibers, and lymphocytes-mediated cytotoxicity. Intravenous immunoglobulins (IVIGs) administration could represent a suitable immunomodulator with this respect. Here we focus on mechanisms of action of immunoglobulins in muscular dystrophies and inflammatory myopathies highlighting results of IVIGs from pre-clinical and case reports evidences.
Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
(Copyright © 2021 Farini, Villa, Tripodi, Legato and Torrente.)

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