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首页> 外文期刊>Protoplasma: An International Journal of Cell Biology >Renal amyloidosis: an update on diagnosis and pathogenesis
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Renal amyloidosis: an update on diagnosis and pathogenesis

机译：肾淀粉样症：诊断和发病机制的更新

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摘要

Amyloidosis is a diverse group of protein conformational disorder which is caused by accumulation and deposition of insoluble protein fibrils in vital tissues or organs, instigating organ dysfunction. Renal amyloidosis is characterized by the acellular Congo red-positive pathologic deposition of amyloid fibrils within glomeruli and/or the interstitium. It is generally composed of serum amyloid A-related protein or an immunoglobulin light chain; other rare forms lysozyme, gelsolin, fibrinogen alpha chain, transthyretin, apolipoproteins AI/AII/AIV/CII/CIII; and the recently identified form ALECT2. This disease typically manifests with heavy proteinuria, nephrotic syndrome, and finally progression to end-stage renal failure. Early diagnosis of renal amyloidosis is arduous as its symptoms appear in later stages with prominent amyloid deposition. The identification of the correct type of amyloidosis is quite troublesome as it can be confused with another related form. Therefore, the exact typing of amyloid is essential for prognosis, treatment, and correct management of renal amyloidosis. The emanation of new techniques of proteomic analysis, for instance, mass spectroscopy/laser microdissection, has provided greater accuracy in amyloid typing. This in-depth review emphasizes on the clinical features, renal pathological findings, and diagnosis of the AL and non-AL forms of renal amyloidosis.
机译：淀粉样蛋白化是一种不同的蛋白质构象紊乱，这是由于在重要组织或器官中的不溶性蛋白原纤维的积累和沉积引起的，煽动器官功能障碍。肾淀粉样蛋白化的特征在于肾小球和/或间隙内的淀粉样蛋白原纤维的细胞刚果红阳性病理沉积。通常由血清淀粉样蛋白A相关蛋白或免疫球蛋白轻链组成;其他稀有形式溶菌酶，凝溶胶素，纤维蛋白原α链，Transthyretin，载脂蛋白AI / AII / AIV / CII / CIII;和最近鉴定的形式2。这种疾病通常表现出重质蛋白尿，肾病综合征，最后进展到终末期肾功能衰竭。早期诊断肾淀粉样症状症状仍然艰巨，因为其症状出现在以后突出的淀粉样蛋白沉积的阶段。鉴定正确类型的淀粉样蛋白病是非常麻烦的，因为它可以与另一种相关形式混淆。因此，淀粉样蛋白的确切键入对于预后，治疗和正确管理肾淀粉样蛋白病的必不可少。蛋白质组学分析新技术的发射，例如质量光谱/激光微粉，在淀粉样蛋白键入方面提供了更高的准确性。这种深入的综述强调了Al和非Al形式的肾淀粉样症状的临床特征，肾病病理发现和诊断。

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