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.

轻链或AL淀粉样变性是德国最丰富的全身性淀粉样变性类型之一。晚期肾脏或心脏参与会导致透析或死亡。该疾病是由抗体轻链的错误折叠和聚集引起的，抗体光链是在单克隆B细胞障碍过程中产生的。 Al淀粉样变性的一个特殊特征是其临床表现的变异性。这种异质性可能是由光链的自然多样性和多态性骨料结构引起的。但是，确切的关系还没有理解。该研究部门旨在阐明两种特殊的突出临床形式的Al淀粉样变性（主要心脏或肾脏受累）如何由蛋白质生化特性引起疾病和临床表现。我们将特别研究序列和一级结构，蛋白水解处理，折叠和多态骨料结构。广泛的方法论支持我们的研究策略，该策略主要旨在改善基本理解，但也可能会对患者产生直接利益，例如在早期诊断中。