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Cell death mechanisms in a mouse model of retinal degeneration in Spinocerebellar ataxia 7
In this study, we show that in a retinal SCA7 mouse model, polyQ ataxin7 induces stress within the retina and activates Muller cells. Moreover, Unfolded Protein Response and autophagy are activated in SCA7 photoreceptors. We have also shown that the photoreceptor death does not involve a caspase-dependent apoptosis but instead involves apoptosis inducing factor (AIF) and Leukocyte Elastase Inhibitor (LEI/L-DNase II). When these two cell death effectors are downregulated by their siRNA, a significant reduction of photoreceptor death is observed. These results highlight the consequences of polyQ protein expression in the ret...
Source: Neuroscience - January 6, 2019 Category: Neuroscience Source Type: research

Mesenchymal Stem Cell-Derived Factors Restore Function to Human Frataxin-Deficient Cells.
Abstract Friedreich's ataxia is an inherited neurological disorder characterised by mitochondrial dysfunction and increased susceptibility to oxidative stress. At present, no therapy has been shown to reduce disease progression. Strategies being trialled to treat Friedreich's ataxia include drugs that improve mitochondrial function and reduce oxidative injury. In addition, stem cells have been investigated as a potential therapeutic approach. We have used siRNA-induced knockdown of frataxin in SH-SY5Y cells as an in vitro cellular model for Friedreich's ataxia. Knockdown of frataxin protein expression to levels de...
Source: Cerebellum - April 29, 2017 Category: Neuroscience Authors: Kemp K, Dey R, Cook A, Scolding N, Wilkins A Tags: Cerebellum Source Type: research