Department of Cellular and Molecular Biology, Centro de Investigaciones Biológicas Margarita Salas CIB-CSIC, 28040, Madrid, Spain; Department of Developmental and Molecular Biology & Institute for Aging Studies, Albert Einstein College of Medicine, Bronx, NY, 10461, USA.
Department of Medicine, Marion Bessin Liver Research Center & Institute for Aging Studies, Albert Einstein College of Medicine, Bronx, NY, 10461, USA.
Mol Aspects Med. 2021 Dec;82:101025. doi: 10.1016/j.mam.2021.101025. Epub 2021 Oct 7.
Chaperone-mediated autophagy (CMA) is a proteolytic process whereby selected intracellular proteins are degraded inside lysosomes. Owing to its selectivity, CMA participates in the modulation of specific regulatory proteins, thereby playing an important role in multiple cellular processes. Studies conducted over the last two decades have enabled the molecular characterization of this autophagic pathway and the design of specific experimental models, and have underscored the importance of CMA in a range of physiological processes beyond mere protein quality control. Those findings also indicate that decreases in CMA function with increasing age may contribute to the pathogenesis of age-associated diseases, including neurodegeneration and cancer. In the context of neurological diseases, CMA impairment is thought to contribute to the accumulation of misfolded/aggregated proteins, a process central to the pathogenesis of neurodegenerative diseases. CMA therefore constitutes a potential therapeutic target, as its induction accelerates the clearance of pathogenic proteins, promoting cell survival. More recent evidence has highlighted the important and complex role of CMA in cancer biology. While CMA induction may limit tumor development, experimental evidence also indicates that inhibition of this pathway can attenuate the growth of established tumors and improve the response to cancer therapeutics. Here, we describe and discuss the evidence supporting a role of impaired CMA function in neurodegeneration and cancer, as well as future research directions to evaluate the potential of this pathway as a target for the prevention and treatment of these diseases.
伴侣蛋白介导的自噬(CMA)是一种蛋白水解过程,其中选定的细胞内蛋白在溶酶体内部被降解。由于其选择性,CMA 参与了特定调节蛋白的调节,从而在多种细胞过程中发挥重要作用。过去二十年的研究使人们能够对这种自噬途径进行分子特征描述,并设计出特定的实验模型,同时强调了 CMA 在许多生理过程中的重要性,不仅仅局限于蛋白质质量控制。这些发现还表明,随着年龄的增长,CMA 功能的下降可能导致与年龄相关疾病的发病机制,包括神经退行性疾病和癌症。在神经疾病的背景下,CMA 功能障碍被认为有助于错误折叠/聚集蛋白的积累,这是神经退行性疾病发病机制的核心过程。因此,CMA 构成了一个潜在的治疗靶点,因为它的诱导可以加速致病性蛋白的清除,促进细胞存活。最近的证据强调了 CMA 在癌症生物学中的重要而复杂的作用。虽然 CMA 的诱导可能会限制肿瘤的发展,但实验证据也表明,抑制该途径可以减弱已建立的肿瘤的生长,并改善对癌症治疗的反应。在这里,我们描述并讨论了支持 CMA 功能障碍在神经退行性疾病和癌症中的作用的证据,以及评估该途径作为预防和治疗这些疾病的靶点的潜在未来研究方向。