Trost Maja, Carbon Maren, Edwards Christine, Ma Yilong, Raymond Deborah, Mentis Marc J, Moeller James R, Bressman Susan B, Eidelberg David
Center for Neurosciences, North Shore-Long Island Jewish Research Institute, 350 Community Drive, Manhasset, NY 11030, USA.
Ann Neurol. 2002 Dec;52(6):853-6. doi: 10.1002/ana.10418.
The DYT1 dystonia mutation is associated with an abnormal metabolic brain network characterized by hypermetabolism of the basal ganglia, supplementary motor area, and the cerebellum. In this study, we quantified the activity of this network in carriers of other dystonia mutations to determine whether this functional abnormality is linked to genotype. The findings suggest that the DYT1 metabolic topography is not genotype specific and may be present in carriers of other dystonia mutations.
DYT1肌张力障碍突变与一种异常的脑代谢网络相关,其特征为基底神经节、辅助运动区和小脑的代谢亢进。在本研究中,我们对其他肌张力障碍突变携带者中该网络的活动进行了量化,以确定这种功能异常是否与基因型有关。研究结果表明,DYT1代谢地形图并非基因型特异性的,可能存在于其他肌张力障碍突变的携带者中。