Human α-synuclein-harboring familial Parkinson's disease-linked Ala-53 → Thr mutation causes neurodegenerative disease with α-synuclein aggregation in transgenic mice

By Michael K. Lee, Wanda Stirling, Yanqun Xu, Xueying Xu, D. Qui, A. Mandir, T. Dawson, N. Copeland, Nancy A. Jenkins, D. Price. Proceedings of the National Academy of Sciences of the United States of America, 2002

745 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Cognitive Neuroscience, Neurology

View Paper: https://www.semanticscholar.org/paper/f64c2dca8b9c9a49686e126f24f370496acd53d5

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In Cellular and Molecular Neuroscience

In Cognitive Neuroscience

Mark P. Mattson

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Citations in the corpus

Calcium and neurodegeneration (2007, background): “For example, dietary energy restriction (Lee et al., 2002a,b; Maswood et al., 2004) and exercise (Smith & Zigmond, 2003; Vaynman & Gomez-Pinilla, 2006) can increase brain-derived neurotrophic factor (BDNF) and glial cell line-derived neuro- trophic factor (GDNF) signaling.”
Sox2 deficiency causes neurodegeneration and impaired neurogenesis in the adult mouse brain (2004, background): “It should be considered that the vulnerability of these neurones to damage (and aggregate formation) appears to be extremely reduced in mice versus humans (Lee et al., 2002).”
Genetic Animal Models of Parkinson’s Disease (2010, positive): “Fibrils of a-synuclein also play a role in the toxicity since the formation of a-synuclein aggregates is required for significant neurodegeneration (Giasson et al., 2002; Lee et al., 2002; Periquet et al., 2007).”
Parkinson's Disease Mechanisms and Models (2003, background): “Nevertheless, this model was the first to link an environmental toxin of possible relevance to PD to the pathologic hallmark of -synuclein aggregation, an association also seen in cell culture studies (Uversky et al., 2001; Sherer et al., 2002; Lee et al., 2002a).”
Intravesicular Localization and Exocytosis of α-Synuclein and its Aggregates (2005, methodology): “Although majority of cellular -syn is present in the cytosol, a small portion is also present in membranous vesicle fractions in tissue and cell homogenates (George et al., 1995; H.-J. Lee et al., 2002a).”
Molecular pathophysiology of Parkinson's disease. (2005, background): “However, the failure of transgenic mice overexpressing the protofibrillogenic A30P mutant α-synuclein to exhibit neurodegeneration (Lee et al. 2002) suggests that protofibrils may not be the primary cytotoxic moiety.”
Parkinson's disease: genetics and pathogenesis. (2011, background): “Models based on overexpression of α-synuclein that carry the diseasecausing A53T mutation show marked aggregation of α-synuclein in the brainstem and spinal cord, along with severe axonopathy and related motor deficits (130, 131).”