{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T16:14:28Z","timestamp":1762100068792},"reference-count":38,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2003,5,13]],"date-time":"2003-05-13T00:00:00Z","timestamp":1052784000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2003,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cystatin C (CC), an endogenous cysteine protease inhibitor, is accumulated within amyloid\u2010\u03b2 (A\u03b2) amyloid deposits in Alzheimer's disease (AD) brain and was proposed to play a role in the AD pathogenesis. Because the chemo\u2010morphologic muscle phenotype of sporadic inclusion\u2010body myositis (s\u2010IBM) has several similarities with the phenotype of AD brain, including abnormal accumulation of A\u03b2 deposits, we studied expression and localization of CC in muscle biopsies of 10\u2003s\u2010IBM, and 16 disease\u2010 and five normal\u2010control muscle biopsies. Physical interaction of CC with amyloid\u2010\u03b2 precursor protein (A\u03b2PP) was studied by a combined immunoprecipitation\/immunoblotting technique in the s\u2010IBM muscle biopsies and in A\u03b2PP\u2010overexpressing cultured human muscle fibers. In all s\u2010IBM muscle biopsies, CC\u2010immunoreactivity either colocalized with, or was adjacent to, the A\u03b2\u2010immunoreactive inclusions in 80\u201390% of the vacuolated muscle fibers, mostly in non\u2010vacuolated regions of their cytoplasm. Ultrastructurally, CC immunoreactivity\u2010colocalized with A\u03b2 on 6\u201310\u2003nm amyloid\u2010like fibrils and floccular material. By immunoblotting, CC expression was strongly increased in IBM muscle as compared to the controls. By immunoprecipitation\/immunoblotting experiments, CC coimmunoprecipitated with A\u03b2PP, both in s\u2010IBM muscle and in A\u03b2PP\u2010overexpressing cultured normal human muscle fibers. Our studies (i) demonstrate for the first time that CC physically associates with A\u03b2PP, and (ii) suggest that CC may play a novel role in the s\u2010IBM pathogenesis, possibly by influencing A\u03b2PP processing and A\u03b2 deposition.<\/jats:p>","DOI":"10.1046\/j.1471-4159.2003.01798.x","type":"journal-article","created":{"date-parts":[[2003,6,3]],"date-time":"2003-06-03T10:18:04Z","timestamp":1054635484000},"page":"1539-1546","source":"Crossref","is-referenced-by-count":32,"title":["Cystatin C colocalizes with amyloid\u2010\u03b2 and coimmunoprecipitates with amyloid\u2010\u03b2 precursor protein in sporadic inclusion\u2010body myositis muscles"],"prefix":"10.1111","volume":"85","author":[{"given":"Gaetano","family":"Vattemi","sequence":"first","affiliation":[]},{"given":"W.","family":"King Engel","sequence":"additional","affiliation":[]},{"given":"Janis","family":"McFerrin","sequence":"additional","affiliation":[]},{"given":"Valerie","family":"Askanas","sequence":"additional","affiliation":[]}],"member":"311","published-online":{"date-parts":[[2003,5,13]]},"reference":[{"key":"e_1_2_21_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrd.1142"},{"key":"e_1_2_21_3_1","first-page":"85","volume-title":"The Handbook of Clinical Neurology, Myopathies","author":"Askanas V.","year":"1992"},{"key":"e_1_2_21_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-200210000-00002"},{"key":"e_1_2_21_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(92)90241-X"},{"key":"e_1_2_21_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410340408"},{"key":"e_1_2_21_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.6.1265-a"},{"key":"e_1_2_21_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390318"},{"key":"e_1_2_21_9_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.3.1314"},{"key":"e_1_2_21_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-199707070-00012"},{"key":"e_1_2_21_11_1","first-page":"889","article-title":"Light and electron microscopic immunolocalization of presenilin 1 in abnormal muscle fibers of patients with sporadic inclusion\u2010body myositis and autosomal\u2010recessive inclusion\u2010body myopathy","volume":"152","author":"Askanas V.","year":"1998","journal-title":"Am. 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