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当前关于淀粉样变性免疫病理学的概念。

Currents concepts on the immunopathology of amyloidosis.

机构信息

Division of Rheumatology, Allergy and Clinical Immunology, Genome and Biomedical Sciences Facility, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA 95616, USA.

出版信息

Clin Rev Allergy Immunol. 2010 Apr;38(2-3):97-106. doi: 10.1007/s12016-009-8163-9.

Abstract

Amyloidosis is defined as the extracellular accumulation at systemic or organ-specific level of insoluble low molecular weight protein fibrils manifesting a beta pleated sheet configuration and a characteristic staining pattern. Several different types of proteins may lead to this phenomenon, and amyloidosis is defined by the biochemical nature of the protein in the deposits and further classified according to whether the deposits are localized or systemic, acquired or inherited, and by the resulting clinical phenotype. Amyloidosis includes subtypes such as light chain, associated with serum amyloid A protein, heritable and familial forms, dialysis-related disease, and organ-specific conditions. The pathogenesis and clinical features of these clinical and pathological entities will be critically discussed in this review article.

摘要

淀粉样变性定义为不溶性低分子量蛋白质纤维在全身或器官特异性水平的细胞外蓄积,表现为β折叠片构型和特征性染色模式。几种不同类型的蛋白质可能导致这种现象,淀粉样变性根据沉积物中蛋白质的生化性质进行定义,并根据沉积物的局部或全身、获得性或遗传性以及由此产生的临床表型进一步分类。淀粉样变性包括与血清淀粉样 A 蛋白相关的轻链、遗传性和家族性形式、透析相关疾病以及器官特异性疾病等亚型。本文将对这些临床和病理实体的发病机制和临床特征进行批判性讨论。

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