Correction: Vascular regression precedes motor neuron loss in the FUS (1-359) ALS mouse model
نویسندگان
چکیده
منابع مشابه
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal adult motor neuron disease. Decreased expression of SMN causes the fatal childhood motor neuron disorder spinal muscular atrophy (SMA). The SMN complex localizes in both the cytoplasm and nuclear Gems, and loss of Gems is a cellular hallmark of fibroblasts in patients with SMA. Here, we report that FUS a...
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Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of-function mechanisms have been proposed to explain how mutant FUS leads to motor neuron degeneration, but neither has been firmly established in the pathogenesis of ALS. Here we characterize a series of transgenic FUS mouse...
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ژورنال
عنوان ژورنال: Disease Models & Mechanisms
سال: 2020
ISSN: 1754-8411,1754-8403
DOI: 10.1242/dmm.045310