Amyotrophic lateral sclerosis: recent insights from genetics and transgenic mice
Article Abstract:
Amyotrophic lateral sclerosis (ALS), an age-dependent neurodegenerative disorder, results from excitotoxic and oxidative injury to subcellular motor neuron targets that leads to motor neuron death. Mutations in the gene encoding the cytosolic copper-zinc SOD in the ALS locus on human chromosome 21q result in ALS. This cytoskeletal protein is essential for motor neuron viability. The excitotoxic cellular injury associated with ALS is mediated by superoxide anion while the development of neuronal toxicity involves nitric oxide. Extracellular factors such as motor neuron trauma or exogenous toxins initiate or promote the several extrinsic cellular processes involved in the pathogenesis of ALS.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1995
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Specific deficit of the ON response in visual transmission by targeted disruption of the mGluR6 gene
Article Abstract:
A study of the function of mGluR6 gene in the ON mechanism in ON bipolar cells through the targeted disruption of mGluR6 reveals that disruption of the gene leads to immunoreactivity and mRNA loss in the gene. The homozygous mice do not exhibit any developmental or behavioral defects. ERG analysis and superior colliculus recordings reveal that ON responses are totally eliminated by mGluR6, without altering the visual transmission OFF responses.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1995
User Contributions:
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