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通过沉降速度分析型超速离心法追踪病理游离轻链中还原诱导的分子变化。

Tracking reduction-induced molecular changes in pathological free light chains by SV-AUC.

作者信息

Tucholski Florian T, Sternke-Hoffmann Rebecca, Pauly Thomas, Norrild Rasmus K, Boquoi Amelie, Fenk Roland, Nagel Luitgard, Buell Alexander K, Haas Rainer, Willbold Dieter

机构信息

Institut für Physikalische Biologie, Heinrich Heine University, Düsseldorf, Germany.

PSI Center for Life Sciences, 5232, Villigen, Switzerland.

出版信息

Eur Biophys J. 2025 Aug 5. doi: 10.1007/s00249-025-01788-2.

Abstract

Multiple myeloma is a blood cancer characterized by plasma cell proliferation and excessive production of monoclonal proteins, often leading to renal complications and other forms of organ damage. A set of nine immunoglobulin free light chain (FLC) samples purified from urine of multiple myeloma patients was subjected to sedimentation velocity analysis. Aim of the study was to track changes of the oligomerization state of each FLC while triggering reduction-induced aggregation into larger structures. Sedimentation velocity experiments, combined with further techniques sensitive to structural changes, were performed to determine the degree of FLC oligomerization in each patient sample under different experimental conditions. Structurally, the FLC monomers are stabilized by two intramolecular disulfide bonds, while covalent dimerization occurs through an unpaired C-terminal cysteine residue. Incubation with the reducing agent TCEP cleaves intra- and intermolecular disulfide bonds, destabilizing both monomers and dimers. Remarkably, different incubation times revealed that destabilized dimers do not dissociate into stable monomers but instead accumulate directly into oligomers and higher-order aggregates. In addition to larger aggregates, fragments with sizes around 1 S were detected with increasing TCEP incubation time. This fragmentation behavior was consistent among FLCs originating from the immunoglobulin kappa variable 1-33 gene (IGKV1-33). Sedimentation velocity-based characterization of FLCs can provide insights into the relationship between their stability and aggregation capacity. An understanding of this relationship is crucial for the development of therapeutic strategies to prevent renal complications associated with monoclonal gammopathies such as multiple myeloma.

摘要

多发性骨髓瘤是一种血液癌症,其特征为浆细胞增殖和单克隆蛋白的过度产生,常导致肾脏并发症和其他形式的器官损伤。从多发性骨髓瘤患者尿液中纯化出的一组九个免疫球蛋白游离轻链(FLC)样本进行了沉降速度分析。该研究的目的是追踪每个FLC的寡聚化状态变化,同时引发还原诱导的聚合成更大结构的过程。进行了沉降速度实验,并结合对结构变化敏感的其他技术,以确定在不同实验条件下每个患者样本中FLC的寡聚化程度。在结构上,FLC单体通过两个分子内二硫键稳定,而共价二聚化通过一个未配对的C末端半胱氨酸残基发生。用还原剂三(2-羧乙基)膦(TCEP)孵育会裂解分子内和分子间的二硫键,使单体和二聚体均不稳定。值得注意的是,不同的孵育时间表明,不稳定的二聚体不会解离成稳定的单体,而是直接聚集成寡聚体和高阶聚集体。除了更大的聚集体外,随着TCEP孵育时间的增加,还检测到大小约为1 S的片段。这种片段化行为在源自免疫球蛋白κ可变区1-33基因(IGKV1-33)的FLC中是一致的。基于沉降速度的FLC表征可以深入了解其稳定性与聚集能力之间的关系。了解这种关系对于制定预防与多发性骨髓瘤等单克隆丙种球蛋白病相关的肾脏并发症的治疗策略至关重要。

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