Ramamohan Priti Y, Gourie-Devi M, Nalini A, Shobha K, Ramamohan Y, Joshi Preeti, Raju T R
Department of Neurophysiology, National Institute of Mental Health and Neuro Sciences, Bangalore, India.
Amyotroph Lateral Scler. 2007 Apr;8(2):79-82. doi: 10.1080/08037060601145489.
We have previously shown in our laboratory that cerebrospinal fluid from ALS patients (ALS-CSF) contains putative toxic factor(s). In the present study we determined the effect of ALS-CSF on the integrity of the Golgi apparatus of spinal motor neurons in the neonatal rats. CSF was injected intrathecally into three-day-old rat pups and subsequently the ultrastructural changes in the motor neurons were studied after 48 h, 1, 2 and 3 weeks. We observed that ALS-CSF causes fragmentation of the Golgi apparatus in a considerable number of motor neurons in the spinal cord. This was further confirmed when motor neurons were stained with an antibody against a medial Golgi protein (MG160). Thus, we suggest that the putative toxin(s) present in ALS-CSF may cause impairment in the protein processing leading to motor neuron death.
我们之前在实验室中已表明,肌萎缩侧索硬化症患者的脑脊液(ALS-CSF)含有假定的毒性因子。在本研究中,我们测定了ALS-CSF对新生大鼠脊髓运动神经元高尔基体完整性的影响。将脑脊液鞘内注射到三日龄的幼鼠体内,随后在48小时、1周、2周和3周后研究运动神经元的超微结构变化。我们观察到,ALS-CSF会导致脊髓中相当数量的运动神经元高尔基体碎片化。当用针对高尔基体中间蛋白(MG160)的抗体对运动神经元进行染色时,这一点得到了进一步证实。因此,我们认为ALS-CSF中存在的假定毒素可能会导致蛋白质加工受损,从而导致运动神经元死亡。