TDP-43 in inclusion body myositis

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منابع مشابه

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.

The nucleic acid binding protein TDP-43 was recently identified in normal myonuclei and in the sarcoplasm of inclusion body myositis (IBM) muscle. Here we found TDP-43 sarcoplasmic immunoreactivity in 23% of IBM myofibers, while other reported IBM biomarkers were less frequent, with rimmed vacuoles in 2.8%, fluorescent Congo red material in 0.57%, SMI-31 immunoreactivity in 0.83%, and focal R12...

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TDP-43: a reliable immunohistochemistry marker for inclusion body myositis?

TDP-43 is a 414 amino acid protein encoded by the transactive response (TAR) DNA binding protein gene on chromosome 1p36. It was first cloned as a human protein capable of binding to the TAR DNA of HIV-1.11 It was later identified as part of a complex involved in splicing of the cystic fibrosis transmembrane conductance regulator gene.3 TDP-43 is highly conserved, ubiquitously expressed and pre...

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Inclusion-body myositis

Inclusion-body myositis is the most frequent and disabling myopathy seen in patients over 50 years of age. The distinct clinical features that lead to correct diagnosis and inclusion-body myositis mimics are highlighted. Inclusionbody myositis has a complex pathogenesis in which autoimmune and inflammatory features coexist with elements of degeneration and abundant accumulations of various stre...

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Inclusion Body Myositis

Inclusion body myositis is an insidious, slowly progressive myopathy of middle-aged and older individuals. Because of these characteristics, diagnosis is often delayed. Affected muscle is marked by the presence of rimmed vacuoles, inclusions, and an inflammatory infiltrate largely made up of CD8 T lymphocytes and macrophages. The inclusions contain beta-amyloid and phosphorylated tau protein, a...

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Inclusion body myositis.

The histochemical and ultrastructural study of muscle biopsies of two patients with a chronic muscle weakness and wasting showed particular changes in muscle fibers: (1) peripheral lined vacuoles, containing whorls of membranes and cytoplasmic debris; (2) collections of intranuclear and intrasarcoplasmic tubular filaments (16-18 nm in external diameter and 6.5 nm in inner diameter). These chang...

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ژورنال

عنوان ژورنال: Nature Reviews Neurology

سال: 2009

ISSN: 1759-4758,1759-4766

DOI: 10.1038/nrneurol.2009.124