Université Côte d'Azur, Centre National de la Recherche Scientifique, Institut de Pharmacologie Moléculaire et Cellulaire, Valbonne Sophia Antipolis, France.
Université Côte d'Azur, Centre National de la Recherche Scientifique, Institut de Pharmacologie Moléculaire et Cellulaire, Valbonne Sophia Antipolis, France.
Kidney Int. 2023 Feb;103(2):251-253. doi: 10.1016/j.kint.2022.10.020.
The major form of membranous nephropathy is characterized by autoantibodies to phospholipase A2 receptor 1 (PLA2R1). The study by Tomas et al. describes the first animal model where human PLA2R1 is ectopically expressed in mouse podocytes. Intriguingly, the transgenic mice spontaneously develop anti-human PLA2R1 antibodies and membranous nephropathy-like features, including immune deposits and nephrotic syndrome. The model raises questions about the spontaneous production of anti-human PLA2R1 antibodies and the additional steps to establish a bona fide animal model of membranous nephropathy.
主要形式的膜性肾病的特点是自身抗体磷脂酶 A2 受体 1 (PLA2R1)。托马斯等人的研究描述了第一个动物模型，其中人 PLA2R1 在小鼠足细胞中异位表达。有趣的是，转基因小鼠自发产生抗人 PLA2R1 抗体和膜性肾病样特征，包括免疫沉积物和肾病综合征。该模型提出了关于自发产生抗人 PLA2R1 抗体的问题，以及建立膜性肾病真正动物模型的其他步骤。
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