PMID: 12787072Jun 6, 2003Paper

Cystatin C colocalizes with amyloid-beta and coimmunoprecipitates with amyloid-beta precursor protein in sporadic inclusion-body myositis muscles

Journal of Neurochemistry
Gaetano VattemiValerie Askanas

Abstract

Cystatin C (CC), an endogenous cysteine protease inhibitor, is accumulated within amyloid-beta (A beta) amyloid deposits in Alzheimer's disease (AD) brain and was proposed to play a role in the AD pathogenesis. Because the chemo-morphologic muscle phenotype of sporadic inclusion-body myositis (s-IBM) has several similarities with the phenotype of AD brain, including abnormal accumulation of A beta deposits, we studied expression and localization of CC in muscle biopsies of 10 s-IBM, and 16 disease- and five normal-control muscle biopsies. Physical interaction of CC with amyloid-beta precursor protein (A beta PP) was studied by a combined immunoprecipitation/immunoblotting technique in the s-IBM muscle biopsies and in A beta PP-overexpressing cultured human muscle fibers. In all s-IBM muscle biopsies, CC-immunoreactivity either colocalized with, or was adjacent to, the A beta-immunoreactive inclusions in 80-90% of the vacuolated muscle fibers, mostly in non-vacuolated regions of their cytoplasm. Ultrastructurally, CC immunoreactivity-colocalized with A beta on 6-10 nm amyloid-like fibrils and floccular material. By immunoblotting, CC expression was strongly increased in IBM muscle as compared to the controls. By immunoprecipitat...Continue Reading

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Citations

Sep 6, 2008·Molecular Neurobiology·Daniela L RebolledoNibaldo C Inestrosa
Jun 24, 2004·Neurobiology of Aging·Magdalena SastreEfrat Levy
Dec 26, 2003·The American Journal of Pathology·Gaetano VattemiValerie Askanas
Oct 6, 2007·Current Opinion in Rheumatology·Valerie Askanas, W King Engel
Jul 23, 2009·BMJ : British Medical Journal·Steven A Greenberg
Feb 23, 2010·Journal of Health Care for the Poor and Underserved·Josiah Ochieng, Gautam Chaudhuri
May 15, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Masashi KitazawaFrank M LaFerla
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May 28, 2020·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Lidia CicconeNicolo Tonali
May 5, 2004·Biochemistry·Keisuke WakasugiIsao Morishima

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