ACCUMULATION OF HIPPOCAMPAL CHOLINERGIC NEUROSTIMULATING PEPTIDE (HCNP)-PRECURSOR PROTEIN IN MUSCLE FIBERS IN INCLUSION-BODY MYOSITIS

概要

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In* inclusion-body myositis (IBM), muscle fibers show abnormal accumulations of several molecules that are also deposited in the Alzheimer's disease brain. We have previously demonstrated that hippocampal cholinergic neurostimulating peptide (HCNP) enhances cholinergic phenotype development, and that HCNP-related antigen accumulates specifically in Hirano bodies. To examine whether HCNP-precursor protein is involved in IBM molecular pathogenesis, we employed immunocytochemical and Western blot analysis. Immunocytochemical analysis showed that in all degenerated muscles from sIBM patients, but not in muscles from non-IBM disease controls, the antigens were specifically precipitated within the inclusions and cytoplasm of vacuolated or vacuole-free atrophic fibers, mostly co-localized with ubiquitin. Furthermore, Western blot analysis indicated that in sIBM muscle HCNP-precursor protein was specifically increased in the insoluble fraction.
名古屋市立大学の論文
2002-04-30