Chiseko Ikenaga

Keywords VCP nuclear pore complex nucleoporin TDP-43 nucleocytoplasmic transport protein quality control ALS Drosophila neurodegeneration muscle disease Get full text access Log in, subscribe or purchase for full access. References 1. Lin, D.H. ∙ Hoelz, A. The Structure of the Nuclear Pore Complex (An Update) Annu. Rev. Biochem. 2019; 88:725-783 2. D'Angelo, M.A. ∙ Raices, M. ∙ Panowski, S.H. ...

Keywords muscle inflammation idiopathic inflammatory myopathy inclusion body myositis dermatomyositis immune-mediated necrotizing myopathy antisynthetase syndrome Inflammatory myopathies: where do we stand? IIMs, commonly referred to as myositis, represent a heterogeneous group of rare diseases characterized by immune-mediated muscle damage and varying clinical presentations.

Abstract Inclusion body myositis (IBM) is the most prevalent inflammatory muscle disease in older adults with no effective therapy available. In contrast to other inflammatory myopathies such as subacute, immune-mediated necrotizing myopathy (IMNM), IBM follows a chronic disease course with both inflammatory and degenerative features of pathology. Moreover, causal factors and molecular drivers of IBM progression are largely unknown.