SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments

@article{Joyce2011SOD1AT,
  title={SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments},
  author={Peter I. Joyce and P. Fratta and E. Fisher and A. Acevedo-Arozena},
  journal={Mammalian Genome},
  year={2011},
  volume={22},
  pages={420-448}
}
  • Peter I. Joyce, P. Fratta, +1 author A. Acevedo-Arozena
  • Published 2011
  • Biology, Medicine
  • Mammalian Genome
  • Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease with no cure. Breakthroughs in understanding ALS pathogenesis came with the discovery of dominant mutations in the superoxide dismutase 1 gene (SOD1) and other genes, including the gene encoding transactivating response element DNA binding protein-43 (TDP-43). This has led to the creation of animal models to further our understanding of the disease and identify a number of ALS-causing mechanisms, including mitochondrial… CONTINUE READING
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    References

    SHOWING 1-10 OF 232 REFERENCES
    TDP-43-Based Animal Models of Neurodegeneration: New Insights into ALS Pathology and Pathophysiology
    • 79
    • Highly Influential
    Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis
    • 19
    Amyotrophic lateral sclerosis associated with mutations in the CuZn superoxide dismutase gene
    • P. Andersen
    • Medicine, Biology
    • Current neurology and neuroscience reports
    • 2006
    • 306
    Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis
    • Y. Guo, D. Wu, +6 authors C. Li
    • Biology, Medicine
    • Experimental & Molecular Medicine
    • 2009
    • 52
    • PDF
    A Drosophila Model for Amyotrophic Lateral Sclerosis Reveals Motor Neuron Damage by Human SOD1*♦
    • 136
    • Highly Influential
    • PDF
    Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching
    • 112
    • Highly Influential
    Mitochondrial dysfunction is a converging point of multiple pathological pathways in amyotrophic lateral sclerosis.
    • 78
    • PDF
    The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies
    • M. Gurney
    • Medicine, Biology
    • Journal of the Neurological Sciences
    • 1997
    • 164
    Ubiquilin Modifies TDP-43 Toxicity in a Drosophila Model of Amyotrophic Lateral Sclerosis (ALS)*
    • 119
    • Highly Influential
    • PDF