Röcken C
Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Christian-Albrechts-Universität Kiel, Arnold-Heller-Straße 3/14, 24105, Kiel, Deutschland.
Pathologe. 2018 Dec;39(Suppl 2):301-305. doi: 10.1007/s00292-018-0515-x.
Amyloid is a pathologic fibrillar aggregation of polypeptides in a cross-β-sheet conformation. Amyloidoses are caused by the deposition of amyloid and may occur as cerebal and extracerebral diseases. A total of 35 different amyloid proteins have been identified. The Amyloid Registry Kiel was founded in 2009 at the Department of Pathology of the Christian-Albrechts-University Kiel. It currently houses more than 6700 cases of diverse anatomical origin with histologically confirmed amyloid and amyloidosis. This large series of cases provides unique insights into an otherwise rare disease. This includes the validation of novel dyes for the histological recognition and diagnosis of amyloid (luminescent conjugated polythiophenes), the in-depth analysis of amyloid associated with carpal tunnel syndrome, the distribution pattern of diverse amyloids in biopsies of the gastrointestinal tract and the prognostic significance of immunoglobulin light chain-derived AL amyloid load in cardiac biopsies. Finally, the application of MALDI (matrix-assisted laser desorption/ionization) mass spectrometry imaging provided valuable insights into the complexity of amyloid deposits, which consist of a diagnostic disease-specific and amyloid-specific protein/peptide mixture.
淀粉样蛋白是一种呈交叉β折叠构象的病理性多肽纤维聚集体。淀粉样变性由淀粉样蛋白沉积引起，可表现为脑部和脑外疾病。已鉴定出总共35种不同的淀粉样蛋白。基尔淀粉样蛋白登记处于2009年在基尔基督教-阿尔布雷希茨大学病理学系成立。它目前收纳了6700多例来自不同解剖学部位、经组织学证实为淀粉样蛋白和淀粉样变性的病例。这一大系列病例为这种原本罕见的疾病提供了独特的见解。这包括用于淀粉样蛋白组织学识别和诊断的新型染料（发光共轭聚噻吩）的验证、与腕管综合征相关的淀粉样蛋白的深入分析、胃肠道活检中各种淀粉样蛋白的分布模式以及心脏活检中免疫球蛋白轻链衍生的AL淀粉样蛋白负荷的预后意义。最后，基质辅助激光解吸/电离（MALDI）质谱成像的应用为淀粉样沉积物的复杂性提供了有价值的见解，这些沉积物由具有诊断疾病特异性和淀粉样蛋白特异性的蛋白质/肽混合物组成。
