Centre for Molecular Medicine and Therapeutics, British Columbia Children's Hospital Research Institute, University of British Columbia, Vancouver, BC V5Z 4H4, Canada.
Department of Medical Genetics, Faculty of Medicine, University of British Columbia, Vancouver, BC V5Z 4H4, Canada.
Int J Mol Sci. 2021 Mar 12;22(6):2905. doi: 10.3390/ijms22062905.
The conserved VPS13 proteins constitute a new family of lipid transporters at membrane contact sites. These large proteins are suspected to bridge membranes and form a direct channel for lipid transport between organelles. Mutations in the 4 human homologs () are associated with a number of neurological disorders, but little is known about their precise functions or the relevant contact sites affected in disease. In contrast, yeast has a single Vps13 protein which is recruited to multiple organelles and contact sites. The yeast model system has proved useful for studying the function of Vps13 at different organelles and identifying the localization determinants responsible for its membrane targeting. In this review we describe recent advances in our understanding of VPS13 proteins with a focus on yeast research.
保守的 VPS13 蛋白在膜接触位点构成了一个新的脂质转运蛋白家族。这些大型蛋白被怀疑在细胞器之间形成膜桥并形成脂质运输的直接通道。4 个人类同源物 () 的突变与许多神经退行性疾病有关，但对其确切功能或疾病中受影响的相关接触点知之甚少。相比之下，酵母只有一种 Vps13 蛋白，它被招募到多个细胞器和接触点。酵母模型系统已被证明可用于研究 Vps13 在不同细胞器中的功能，并确定负责其膜靶向的定位决定因素。在这篇综述中，我们描述了对 VPS13 蛋白的理解的最新进展，重点是酵母研究。
