2021
DOI: 10.1038/s41419-021-04404-1
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Autophagy in Spinocerebellar ataxia type 2, a dysregulated pathway, and a target for therapy

Abstract: Spinocerebellar ataxia type 2 (SCA2) is an incurable and genetic neurodegenerative disorder. The disease is characterized by progressive degeneration of several brain regions, resulting in severe motor and non-motor clinical manifestations. The mutation causing SCA2 disease is an abnormal expansion of CAG trinucleotide repeats in the ATXN2 gene, leading to a toxic expanded polyglutamine segment in the translated ataxin-2 protein. While the genetic cause is well established, the exact mechanisms behind neuronal… Show more

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Cited by 23 publications
(21 citation statements)
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“…In addition to changes in GFAP expression and motor neuron counts, both males and females demonstrate increased evidence of the pro-apoptotic marker cleaved caspase-3 activity within the lumbar spinal cord as measured by cytosolic puncta accumulation over time 37 39 , correlating with some of the neuron loss observed in the spinal cord (Fig. 3 G,H).…”
Section: Resultsmentioning
confidence: 92%
“…In addition to changes in GFAP expression and motor neuron counts, both males and females demonstrate increased evidence of the pro-apoptotic marker cleaved caspase-3 activity within the lumbar spinal cord as measured by cytosolic puncta accumulation over time 37 39 , correlating with some of the neuron loss observed in the spinal cord (Fig. 3 G,H).…”
Section: Resultsmentioning
confidence: 92%
“…To study the impact of aging on SCA2 pathology, we used a recently developed striatal SCA2 lentiviral mouse model [ 32 ]. Briefly, we injected wild-type ataxin-2 (WTAtxn2) in the left hemisphere of the striatum and MUTAtxn2 in the contralateral hemisphere, of 3-month-old (3 m) and 18-month-old (18 m) C57BL/6 mice ( Figure 1 A).…”
Section: Resultsmentioning
confidence: 99%
“…On the other hand, neuronal cells are affected by biological aging, which could decisively contribute to disease onset and/or progression. In this work, taking advantage of a recently developed SCA2 lentiviral mouse model [ 32 ], we injected lentiviral vectors encoding for mutant ataxin-2 in 3-month-old (3 m) and in 18-month-old (18 m) mice. We aimed at investigating the impact of aging on SCA2 neuropathological hallmarks associated with the expression of mutant ataxin-2.…”
Section: Introductionmentioning
confidence: 99%
“…In addition to changes in GFAP expression and motor neuron counts, both males and females demonstrate increased evidence of the pro-apoptotic marker cleaved caspase-3 activity within the lumbar spinal cord as measured by cytosolic puncta accumulation over time [37][38][39] , correlating with some of the neuron loss observed in the spinal cord (Fig. 3G, H).…”
Section: Establishment Of a Novel Mouse Model Expressing Gr50mentioning
confidence: 99%