Sporadic ALS Astrocytes Induce Neuronal Degeneration In Vivo
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چکیده
منابع مشابه
Sporadic ALS Astrocytes Induce Neuronal Degeneration In Vivo
Astrocytes from familial amyotrophic lateral sclerosis (ALS) patients or transgenic mice are toxic specifically to motor neurons (MNs). It is not known if astrocytes from sporadic ALS (sALS) patients cause MN degeneration in vivo and whether the effect is specific to MNs. By transplanting spinal neural progenitors, derived from sALS and healthy induced pluripotent stem cells (iPSCs), into the c...
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Recent studies highlight astrocytes as key drivers of motor neuron (MN) degeneration and disease propagation in mutant human superoxide dismutase 1 (mSOD1)-mediated amyotrophic lateral sclerosis. However, in vivo analysis of specific astrocytic influence in amyotrophic lateral sclerosis has proven difficult because mSOD1 is ubiquitously expressed throughout the CNS of rodent models studied. Her...
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BACKGROUND The homogeneous genotype and stereotyped phenotype of a unique familial form of amyotrophic lateral sclerosis (ALS) (patients homozygous for aspartate-to-alanine mutations in codon 90 (homD90A) superoxide dismutase 1) provides an ideal model for studying genotype/phenotype interactions and pathological features compared with heterogeneous apparently sporadic ALS. The authors aimed to...
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H. G. Niessen, F. Angenstein, K. Sander, H-J. Heinze, H. Scheich, S. Vielhaber Department of Neurology II, University of Magdeburg, Magdeburg, LSA, Germany, Special Laboratory Non-Invasive Brain Imaging, Leibniz Institute for Neurobiology, Magdeburg, LSA, Germany Introduction Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a selective loss of motor neurons in...
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ژورنال
عنوان ژورنال: Stem Cell Reports
سال: 2017
ISSN: 2213-6711
DOI: 10.1016/j.stemcr.2017.03.003