Ab'/> AIPL1: A specialized chaperone for the phototransduction effector
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AIPL1: A specialized chaperone for the phototransduction effector

机译：AIPL1：用于光电扫盲效应器的专用伴侣

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AbstractMolecular chaperones play pivotal roles in protein folding, quality control, assembly of multimeric protein complexes, protein trafficking, stress responses, and other essential cellular processes. Retinal photoreceptor rod and cone cells have an unusually high demand for production, quality control, and trafficking of key phototransduction components, and thus, require a robust and specialized chaperone machinery to ensure the fidelity of sensing and transmission of visual signals. Misfolding and/or mistrafficking of photoreceptor proteins are known causes for debilitating blinding diseases. Phosphodiesterase 6, the effector enzyme of the phototransduction cascade, relies on a unique chaperone aryl hydrocarbon receptor (AhR)-interacting protein-like 1 (AIPL1) for its stability and function. The structure of AIPL1 and its relationship with the client remained obscure until recently. This review summarizes important recent advances in understanding the mechanisms underlying normal function of AIPL1 and the protein perturbations caused by pathogenic mutations.Highlights?We summarize the accumulated evidence that AIPL1 is a specialized chaperone of PDE6.?
机译：摘要“>分子伴侣在蛋白质折叠、质量控制、多聚体蛋白质复合物组装、蛋白质运输、应激反应和其他基本细胞过程中起着关键作用。视网膜光感受器视杆细胞和视锥细胞对关键光传导成分的生产、质量控制和运输有着异常高的需求，因此，需要一个强大的专业化陪伴机器以确保视觉信号的感知和传输的保真度。光感受器蛋白的错误折叠和/或误运输是导致致盲性疾病的已知原因。磷酸二酯酶6是光转导级联的效应酶，其稳定性和功能依赖于独特的伴侣芳基烃受体（AhR）-相互作用蛋白样1（AIPL1）。直到最近，AIPL1的结构及其与客户的关系仍不清楚。本文综述了在理解AIPL1正常功能的机制以及致病性突变引起的蛋白质扰动方面的重要进展？“>我们总结了积累的证据，证明AIPL1是PDE6的一种特殊伴侣。？

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