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Showing 1 to 5 of 5 for “"spinocerebellar ataxia type 1"”.

  1. The Role of Microglia and Astrocyte in Spinocerebellar Ataxia Type 1

    Spinocerebellar ataxia type 1 (SCA1) is a fatal dominantly inherited neurodegenerative disease. Even though there has been illuminating work on the effect of the disease-causing protein, a polyQ expanded ATAXIN-1 (ATXN1) on neurons, the relative contribution to disease of glia has been unknown. …

    umn Repository record for The Role of Microglia and Astrocyte in Spinocerebellar Ataxia Type 1 (opens in a new tab)

  2. Intra-Regional Differences in Cerebellar Vulnerability of Spinocerebellar Ataxia Type 1 Mice

    … in cerebellar disease remains an open question. Spinocerebellar Ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease caused by an abnormal expansion of polyglutamine (polyQ) repeats in the ATAXIN1 (ATXN1) gene and characterized by cerebellar degeneration. Recent studies in …

    umn Repository record for Intra-Regional Differences in Cerebellar Vulnerability of Spinocerebellar Ataxia Type 1 Mice (opens in a new tab)

  3. Ataxin-1 in cognition and mood

    Ataxin-1 (ATXN1), the gene mutated in spinocerebellar ataxia type 1 (SCA1), may affect cognition and mood, but much remains unknown, including which brain areas are responsible, whether ATXN1 affects mood in mice, and the mechanisms of these effects. To answer these questions, we characterized …

    umn Repository record for Ataxin-1 in cognition and mood (opens in a new tab)

  4. The role of gene expression and aging in SCA1

    Spinocerebellar ataxia type 1 is an autosomal dominant disorder caused by a CAG repeat expansion encoding a polyglutamine tract, where patients present with a lack of motor coordination including ataxia. The disease is characterized pathologically by loss of Purkinje cells (PCs) in the cerebellar …

    umn Repository record for The role of gene expression and aging in SCA1 (opens in a new tab)

  5. A Role for PI31-Mediated Proteasome Regulation in Proteostasis and Neuronal Health

    … neuronal degeneration in a Drosophila model of spinocerebellar ataxia type 1(SCA1). These results demonstrate a link between NAD+ and proteasomes that may ultimately prove useful for developing interventions that counter the effects of neurodegeneration and allow for an understanding why this …

    rockefeller Repository record for A Role for PI31-Mediated Proteasome Regulation in Proteostasis and Neuronal Health (opens in a new tab)