The neuronal ceroid lipofuscinoses (NCLs), commonly known as Batten disease, belong to a family of neurological disorders that cause blindness, seizures, loss of motor function and cognitive ability, and premature death. There are 13 different subtypes of NCL that are associated with mutations in 13 genetically distinct genes (CLN1-CLN8, CLN10-CLN14). Similar clinical and pathological profiles of the different NCL subtypes suggest that common disease mechanisms may be involved. As a result, there have been many efforts to determine how NCL proteins are connected at the cellular level. A main driving force for NCL research has been the utilization of mammalian and non-mammalian cellular models to study the mechanisms underlying the disease. One non-mammalian model that has provided significant insight into NCL protein function is the social amoeba Dictyostelium discoideum. Accumulated data from Dictyostelium and mammalian cells show that NCL proteins display similar localizations, have common binding partners, and regulate the expression and activities of one another. In addition, genetic models of NCL display similar phenotypes. This review integrates findings from Dictyostelium and mammalian models of NCL to highlight our understanding of the molecular networking of NCL proteins. The goal here is to help set the stage for future work to reveal the cellular mechanisms underlying the NCLs.

中文翻译：

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神经元类固醇脂褐藻糖中的分子网络：哺乳动物模型和社交变形虫Disctyostelium Discoideum的见解。

神经元类固醇脂褐藻糖（NCL），通常称为巴滕病，属于一系列神经系统疾病，会导致失明，癫痫发作，运动功能和认知能力丧失以及过早死亡。NCL有13种不同的亚型，与13个遗传上不同的基因（CLN1-CLN8，CLN10-CLN14）的突变相关。不同NCL亚型的相似临床和病理学特征提示可能涉及常见的疾病机制。结果，已经进行了许多努力来确定NCL蛋白如何在细胞水平上连接。NCL研究的主要推动力是利用哺乳动物和非哺乳动物细胞模型来研究该疾病的潜在机制。一种对哺乳动物的NCL蛋白功能具有重要见解的非哺乳动物模型是社交变形虫阿米巴盘基网柄菌。Dictyostelium和哺乳动物细胞的积累数据表明，NCL蛋白显示相似的定位，具有共同的结合伴侣，并调节彼此的表达和活性。此外，NCL的遗传模型显示相似的表型。这篇综述整合了双歧杆菌和NCL哺乳动物模型的发现，以突出我们对NCL蛋白分子网络的理解。这里的目标是帮助为将来的工作奠定基础，以揭示NCL背后的细胞机制。并调节彼此的表达和活动。此外，NCL的遗传模型显示相似的表型。这篇综述整合了双歧杆菌和NCL哺乳动物模型的发现，以突出我们对NCL蛋白分子网络的理解。这里的目标是帮助为将来的工作奠定基础，以揭示NCL背后的细胞机制。并调节彼此的表达和活动。此外，NCL的遗传模型显示相似的表型。这篇综述整合了双歧杆菌和NCL哺乳动物模型的发现，以突出我们对NCL蛋白分子网络的理解。这里的目标是帮助为将来的工作奠定基础，以揭示NCL背后的细胞机制。