Sanders Paul W
Division of Nephrology, Department of Medicine, Department of Physiology and Biophysics, and the Nephrology Research and Training Center, University of Alabama at Birmingham, and Department of Veterans Affairs Medical Center, Birmingham, Ala., USA.
Contrib Nephrol. 2011;169:262-269. doi: 10.1159/000313959. Epub 2011 Jan 20.
Immunoglobulin light chains are low molecular weight proteins that are filtered through the glomerulus and reabsorbed into the proximal tubular epithelium by binding initially to a heteromeric receptor complex composed of megalin and cubilin. Saturation of this receptor-mediated endocytotic process results in the presence of free light chains in the distal nephron and urine. In the course of metabolism of monoclonal light chains, nephrotoxicity can occur, resulting in clinical manifestations that can include acute kidney injury and progressive chronic kidney disease. Patterns of tubulopathic renal injury include proximal tubular epithelial cell cytotoxicity, tubulointerstitial nephritis and cast nephropathy (also known as 'myeloma kidney'). Research efforts over the past two decades have refined understanding of the molecular mechanisms involved in light chain-mediated tubular injury and are the subject of this review.
免疫球蛋白轻链是低分子量蛋白质，可通过肾小球滤过，并通过最初与由巨膜蛋白和立方蛋白组成的异源受体复合物结合而被近端肾小管上皮细胞重吸收。这种受体介导的内吞过程饱和会导致远端肾单位和尿液中出现游离轻链。在单克隆轻链的代谢过程中，可能会发生肾毒性，导致包括急性肾损伤和进行性慢性肾病在内的临床表现。肾小管病性肾损伤的模式包括近端肾小管上皮细胞细胞毒性、肾小管间质性肾炎和管型肾病（也称为“骨髓瘤肾”）。过去二十年的研究努力深化了对轻链介导的肾小管损伤所涉及分子机制的理解，这也是本综述的主题。
