Publications by authors named "Kailen Polley"
Article Synopsis
- Mitochondrial dysfunction is linked to neurodegenerative diseases like spinocerebellar ataxia type 1 (SCA1), particularly affecting cerebellar function.
- RNA sequencing and other analyses show that SCA1 leads to reduced efficiency in OXPHOS complex I, even though related genes are upregulated as symptoms begin.
- Treatment with succinic acid can help mitigate some of this dysfunction, suggesting potential therapeutic avenues for improving motor function in affected mice.
Mitochondrial dysfunction plays a significant role in the aging process and in neurodegenerative diseases including several hereditary spinocerebellar ataxias and other movement disorders marked by progressive degeneration of the cerebellum. The goal of this protocol is to assess mitochondrial dysfunction in Spinocerebellar ataxia type 1 (SCA1) and assess the efficacy of pharmacological targeting of metabolic respiration via the water-soluble compound succinic acid to slow disease progression. This approach is applicable to other cerebellar diseases and can be adapted to a host of water-soluble therapies.
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