Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies

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منابع مشابه

Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies.

Mutations of the gene encoding four-and-a-half LIM domain 1 (FHL1) are the causative factor of several X-linked hereditary myopathies that are collectively termed FHL1-related myopathies. These disorders are characterized by severe muscle dysfunction and damage. Here, we have shown that patients with idiopathic inflammatory myopathies (IIMs) develop autoimmunity to FHL1, which is a muscle-speci...

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Autoantibodies in Idiopathic Inflammatory Myopathies

Autoimmune response to nuclear and cytoplasmic autoantigens is detected in about 60-80% of patients affected with idiopathic inflammatory myositis such as polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). Some of the serum autoantibodies are shared with other autoimmune diseases (myositis-associated antibodies MAA) and some of them are unique to myositis (myositis-speci...

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[Myositis specific and myositis associated autoantibodies in idiopathic inflammatory myopathies: a serologic study of 46 patients].

OBJECTIVE To characterize serum autoantibody profiles of patients with idiopathic inflammatory myopathies (IIM) by searching for myositis-specific (MSA) and myositis-associated (MAA) antibodies with sensitive and specific laboratory tests. METHODS We tested the sera from 46 Caucasian patients diagnosed as affected with IIM at the Division of Rheumatology of Padova University (21 polimyositis,...

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FHL1 adds some muscle

can bind to either ankyrin in vitro, what makes the channels preferentially bind to ankyrin G in vivo? To answer this question, Bréchet et al. homed in on the precise residues in sodium channels that are required for the interaction with ankyrin G. They pinpointed a sequence of residues that looked like a target for phosphorylation by CK2, and sure enough, it was. CK2 phosphorylated the sodium ...

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Identification of multiple cancer-associated myositis-specific autoantibodies in idiopathic inflammatory myopathies: a large longitudinal cohort study

BACKGROUND Cancer is a significant complication contributing to increased mortality in idiopathic inflammatory myopathies (IIMs), and the association between IIMs and cancer has been extensively reported. Myositis-specific autoantibodies (MSAs) can help to stratify patients into more homogeneous groups and may be used as a biomarker for cancer-associated myositis. In this study, we aimed to sys...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 2015

ISSN: 0021-9738,1558-8238

DOI: 10.1172/jci81031