Unfounded Claims of Improved Functional Outcomes Attributed to Follistatin Gene Therapy in Inclusion Body Myositis

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Pathogenesis and therapy of inclusion body myositis.

PURPOSE OF REVIEW Inclusion body myositis (IBM) is a poorly understood progressive muscle disease of middle and later life. Its dual pathologies of autoimmunity and unexplained myofiber degeneration and loss have been enigmatic since its earliest descriptions over 40 years ago. No reliable effective therapy currently exists for IBM. This review provides an update of current issues in the pathog...

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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: Molecular Therapy

سال: 2017

ISSN: 1525-0016

DOI: 10.1016/j.ymthe.2017.09.002