Coordination Funds

协调基金

• 批准号: 422443988
• 负责人: Professor Dr. Marcus Fändrich
• 金额: --
• 依托单位: Institut für Proteinbiochemie
• 依托单位国家: 德国
• 项目类别: Research Units
• 财政年份: 2019
• 资助国家: 德国
• 起止时间: 2018-12-31 至 2022-12-31
• 项目状态: 已结题
• 来源: https://gepris.dfg.de/gepris/projekt/422443988?language=en
• 关键词: Coordination; Funds

The light chain or AL amyloidosis is one of the most abundant types of systemic amyloidosis in Germany. Advanced kidney or heart involvement can lead to rapid dialysis or death. The disease is caused by the misfolding and aggregation of antibody light chains, which are produced in the course of a monoclonal B-cell disorder. A particular remarkable feature of AL amyloidosis is the variability of its clinical manifestations. This heterogeneity is probably caused by the natural diversity of light chains and of the polymorphic aggregate structure. However, the exact relationships are not so far understood. This research unit aims to clarify for two specifically prominent clinical forms of AL amyloidosis (dominant heart or kidney involvement) how disease and clinical manifestations arise from protein biochemical properties. We will investigate in particular the sequence and primary structure, the proteolytic processing, the folding and the polymorphic aggregate structure. A broad methodological spectrum supports our research strategy which primarily seeks to improve the basic understanding but which may also create direct benefit for patients, for example in the early diagnosis.

轻链淀粉样变性是德国最常见的系统性淀粉样变性之一。晚期肾脏或心脏受累可导致快速透析或死亡。这种疾病是由单克隆b细胞疾病过程中产生的抗体轻链的错误折叠和聚集引起的。AL淀粉样变的一个特别显著的特征是其临床表现的可变性。这种异质性可能是由轻链的自然多样性和多形性聚集体结构引起的。然而，确切的关系到目前为止还不清楚。本研究单元旨在阐明两种特别突出的AL淀粉样变性临床形式（主要累及心脏或肾脏），疾病和临床表现是如何从蛋白质生化特性中产生的。我们将特别研究序列和初级结构，蛋白质水解加工，折叠和多态聚集体结构。广泛的方法范围支持我们的研究策略，主要是为了提高基本的理解，但也可能为患者创造直接的好处，例如在早期诊断中。