Amyotrophic Lateral Sclerosis · 2007 · 39 citations · 14 references
Life SciencesNeurobiologyNeuroscienceMolecular NeuroscienceNeurobiology Of DiseaseNeurodegenerationAmyotrophic Lateral SclerosisCentral Nervous SystemCentral Nervous System ToxicityNervous TissueNeurodegenerative DiseasesNeurodegenerative DisordersAmyotrophic Lateral Sclerosis PathologyBrain ToxicityGolgi Apparatus DysfunctionGolgi Apparatus In NeuronsGolgi Apparatus LocalizationGolgi Apparatus MorphologyGolgi Apparatus StructureProtein Transport Through The Golgi
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.
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Mutations in Dynein Link Motor Neuron Degeneration to Defects in Retrograde Transport
Majid Hafezparast, Rainer Klocke, Christiana Ruhrberg et al. · Science · 2003 · 688 citations · Full text
Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons
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