Serpell Louise C, Benson Merrill, Liepnieks Juris J, Fraser Paul E
Biochemistry, University of Sussex, John Maynard-Smith Building, Falmer, Brighton, UK.
Amyloid. 2007 Sep;14(3):199-203. doi: 10.1080/13506120701461111.
Hereditary fibrinogen amyloidosis is characterized by deposition of amyloid fibrils in renal glomeruli. The subunit protein of the amyloid fibrils is a proteolytic fragment of the fibrinogen Aalpha-chain. To investigate the structure of fibrinogen amyloid, fibrils were extracted from the tissues of a patient and studied by X-ray fiber diffraction and electron microscopy. We have carried out a full structural characterization of amyloid fibrils taken from disease tissue. These studies revealed that ex vivo fibrinogen amyloid fibrils have a cross-beta structure similar to other chemical types of amyloid fibrils.
遗传性纤维蛋白原淀粉样变性的特征是淀粉样纤维在肾小球中沉积。淀粉样纤维的亚基蛋白是纤维蛋白原Aα链的蛋白水解片段。为了研究纤维蛋白原淀粉样蛋白的结构,从一名患者的组织中提取了纤维,并通过X射线纤维衍射和电子显微镜进行研究。我们对取自疾病组织的淀粉样纤维进行了全面的结构表征。这些研究表明,离体纤维蛋白原淀粉样纤维具有与其他化学类型的淀粉样纤维相似的交叉β结构。