Department of Physics E22 (Biophysics), Technische Universität München, James-Franck-Strasse 1, D-85748 Garching/Munich, Germany.
J Biol Chem. 2012 Jul 27;287(31):26278-90. doi: 10.1074/jbc.M111.291898. Epub 2012 May 27.
Spastin is a hexameric ring AAA ATPase that severs microtubules. To see whether the ring complex funnels the energy of multiple ATP hydrolysis events to the site of mechanical action, we investigate here the cooperativity of spastin. Several lines of evidence indicate that interactions among two subunits dominate the cooperative behavior: (i) the ATPase activity shows a sigmoidal dependence on the ATP concentration; (ii) ATPγS displays a mixed-inhibition behavior for normal ATP turnover; and (iii) inactive mutant subunits inhibit the activity of spastin in a hyperbolic dependence, characteristic for two interacting species. A quantitative model based on neighbor interactions fits mutant titration experiments well, suggesting that each subunit is mainly influenced by one of its neighbors. These observations are relevant for patients suffering from SPG4-type hereditary spastic paraplegia and explain why single amino acid exchanges lead to a dominant negative phenotype. In severing assays, wild type spastin is even more sensitive toward the presence of inactive mutants than in enzymatic assays, suggesting a weak coupling of ATPase and severing activity.
Spastin 是一种六聚体环 AAA ATP 酶,可切断微管。为了观察环复合物是否将多个 ATP 水解事件的能量传递到机械作用部位,我们在此研究 spastin 的协同作用。有几条证据表明,两个亚基之间的相互作用主导着协同行为:(i)ATP 酶活性对 ATP 浓度呈 S 形依赖性;(ii)ATPγS 对正常 ATP 周转显示出混合抑制行为;(iii)无活性突变体亚基以双曲线依赖性抑制 spastin 的活性,这是两种相互作用物质的特征。基于邻接相互作用的定量模型很好地拟合了突变体滴定实验,表明每个亚基主要受其一个邻接的影响。这些观察结果与患有 SPG4 型遗传性痉挛性截瘫的患者有关,并解释了为什么单个氨基酸交换会导致显性负表型。在切断实验中,野生型 spastin 比在酶实验中对无活性突变体的存在更为敏感,这表明 ATP 酶和切断活性之间的耦合较弱。