Muscle fiber dysfunction contributes to weakness in inclusion body myositis

Sporadic inclusion body myositis (IBM) is one of the most common acquired muscle disorders in adults over 50 years old [1,2]. Progressive disease is characterized by atrophy and fatty infiltration of muscle tissue, resulting in muscle weakness [3 –5]. In early disease, the quadriceps, deep finger flexors and the pharyngeal muscles are most frequently affected, restricting functional ability and quality of life [6–8]. IBM is the result of a complex and only partially understood interplay between muscle inflammation, degeneration and impai red proteostasis which results in the accumulation of proteins in rimmed vacuoles [9].

Source: Neuromuscular Disorders - March 5, 2019 Category: Neurology Authors: Saskia Lassche, Anke Rietveld, Arend Heerschap, Hieronymus W van Hees, Maria TE Hopman, Nicol C Voermans, Christiaan GJ Saris, Baziel GM van Engelen, Coen AC Ottenheijm Source Type: research