Nadiminti Sravanthi S P, Kamak Madhushree, Koushika Sandhya P
Department of Biological Sciences, Tata Institute of Fundamental Research, Mumbai 400 005, India.
J Genet. 2018 Jul;97(3):753-771.
Phosphoinositides are a class of membrane lipids that are found on several intracellular compartments and play diverse roles inside cells, such as vesicle formation, protein trafficking, endocytosis etc. Intracellular distribution and levels of phosphoinositides are regulated by enzymes that generate and breakdown these lipids as well as other proteins that associate with phosphoinositides. These events lead to differing levels of specific phosphoinositides on different intracellular compartments. At these intracellular locations, phosphoinositides and their associated proteins, such as Rab GTPases, dynamin and BAR domain-containing proteins, regulate a variety of membrane trafficking pathways. Neurodegenerative phenotypes in disorders such as Parkinson's disease (PD) can arise as a consequence of altered or hampered intracellular trafficking. Altered trafficking can cause proteins such as α-synuclein to aggregate intracellularly. Several trafficking pathways are regulated bymaster regulators such as LRRK2,which is known to regulate the activity of phosphoinositide effector proteins. Perturbing either the levels of phosphoinositides or their interactions with different proteins disrupts intracellular trafficking pathways, contributing to phenotypes often observed in disorders such as Alzheimer's or PDs. Thus, studying phosphoinositide regulation and its role in trafficking can give us a deeper understanding of the contribution of disrupted trafficking to neurodegenerative phenotypes.
磷酸肌醇是一类膜脂,存在于多个细胞内区室,在细胞内发挥多种作用,如囊泡形成、蛋白质运输、内吞作用等。磷酸肌醇的细胞内分布和水平受生成和分解这些脂质的酶以及与磷酸肌醇结合的其他蛋白质调控。这些事件导致不同细胞内区室中特定磷酸肌醇的水平不同。在这些细胞内位置,磷酸肌醇及其相关蛋白,如Rab GTP酶、发动蛋白和含BAR结构域的蛋白,调控多种膜运输途径。帕金森病(PD)等疾病中的神经退行性表型可能是细胞内运输改变或受阻的结果。运输改变可导致α-突触核蛋白等蛋白质在细胞内聚集。几种运输途径受主调节因子如LRRK2调控,已知LRRK2可调节磷酸肌醇效应蛋白的活性。干扰磷酸肌醇的水平或其与不同蛋白质的相互作用会破坏细胞内运输途径,导致在阿尔茨海默病或帕金森病等疾病中经常观察到的表型。因此,研究磷酸肌醇调节及其在运输中的作用可以让我们更深入地了解运输紊乱对神经退行性表型的影响。