Intracytoplasmic inclusion bodies of the substantia nigra in myotonic dystrophy. Immunohistochemical observations.

摘要: We recently reported a significantly higher incidence of intracytoplasmic inclusion bodies (IIBs) the substantia nigra in patients with myotonic dystrophy (MyD) than age-matched controls. The changes are, per se, not specific, since small percentage disease and normal controls also showed similar inclusions. To elucidate pathological significance MyD, we studied immunohistochemical characteristics IIBs eight MyD. Many moderately intense immunoreactivity for ubiquitin, microtubule-associated protein (MAP) 1 MAP 2. However, did react any following: anti-neurofilament antibodies (Abs) (68, 160 200 kDa), anti-neuron-specific enolase antibody (Ab), anti-tau Ab, anti-tubulin Abs (alpha beta), anti-paired helical filament anti-actin anti-phosphorylated epitope neurofilaments anti-synaptophysin anti-myelin basic Ab anti-glial fibrillary acidic Ab. Our results suggest that MyD are related to an alteration neuronal cytoskeleton metabolism affecting microtubular proteins conjunction activation ubiquitin proteolytic systems.