Rong Juan, McGuire John R, Fang Zhi-Hui, Sheng Guoqing, Shin Ji-Yeon, Li Shi-Hua, Li Xiao-Jiang
Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
J Neurosci. 2006 May 31;26(22):6019-30. doi: 10.1523/JNEUROSCI.1251-06.2006.
Mutant huntingtin can affect vesicular and receptor trafficking via its abnormal protein interactions, suggesting that impairment of intracellular trafficking may contribute to Huntington's disease. There is growing evidence that huntingtin-associated protein-1 (HAP1) also interacts with microtubule-dependent transporters and is involved in intracellular trafficking. However, it remains unclear how the trafficking of HAP1 is regulated and contributes to neuronal function. Here we report that phosphorylation of HAP1 decreases its association with microtubule-dependent transport proteins dynactin p150 and kinesin light chain and reduces its localization in neurite tips. Suppressing HAP1 expression by RNA interference reduces neurite outgrowth and the level of tropomyosin-related kinase A receptor tyrosine kinase (TrkA), a nerve growth factor receptor whose internalization and trafficking are required for neurite outgrowth. HAP1 maintains the normal level of membrane TrkA by preventing the degradation of internalized TrkA. Mutant huntingtin also reduces the association of HAP1 with dynactin p150 and kinesin light chain and thereby decreases the intracellular level of TrkA. These findings suggest that HAP1 trafficking is critical for the stability of TrkA and neurite function, both of which can be attenuated by mutant huntingtin.
突变型亨廷顿蛋白可通过其异常的蛋白质相互作用影响囊泡和受体运输,这表明细胞内运输受损可能与亨廷顿病有关。越来越多的证据表明,亨廷顿蛋白相关蛋白1(HAP1)也与微管依赖性转运体相互作用,并参与细胞内运输。然而,HAP1的运输如何被调节以及如何影响神经元功能仍不清楚。在此我们报告,HAP1的磷酸化降低了其与微管依赖性运输蛋白动力蛋白p150和驱动蛋白轻链的结合,并减少了其在神经突尖端的定位。通过RNA干扰抑制HAP1的表达会减少神经突生长以及原肌球蛋白相关激酶A受体酪氨酸激酶(TrkA)的水平,TrkA是一种神经生长因子受体,其内化和运输是神经突生长所必需的。HAP1通过防止内化的TrkA降解来维持膜TrkA的正常水平。突变型亨廷顿蛋白也会减少HAP1与动力蛋白p150和驱动蛋白轻链的结合,从而降低细胞内TrkA的水平。这些发现表明,HAP1运输对于TrkA的稳定性和神经突功能至关重要,而这两者均可被突变型亨廷顿蛋白削弱。