Cell Biology Program, The Hospital for Sick Children, and Biochemistry Department, University of Toronto, Toronto, Ontario, M5G 1L7, Canada.
BMC Biol. 2010 Jul 19;8:94. doi: 10.1186/1741-7007-8-94.
Mutation of the protein spartin is a cause of one form of spastic paraplegia. Spartin interacts with ubiquitin ligases of the Nedd4 family, and a recent report in BMC Biology now shows that it acts as an adaptor to recruit and activate the ubiquitin ligase AIP4 onto lipid droplets, leading to the ubiquitination and degradation of droplet-associated proteins. A deficiency of spartin apparently causes lipid droplets to accumulate.
蛋白质 spartin 的突变是痉挛性截瘫的一种形式的病因。Spartin 与 Nedd4 家族的泛素连接酶相互作用,BMC 生物学的一篇最新报道现在表明,它作为衔接蛋白将泛素连接酶 AIP4 募集到脂滴上,从而导致与脂滴相关的蛋白质的泛素化和降解。显然,spartin 的缺乏会导致脂滴积累。