Sirtuin 1 (Sirt1) is a NAD + -dependent deacetylase capable of countering age-related
neurodegeneration, but the basis of Sirt1 neuroprotection remains elusive. Spinocerebellar ataxia …

Cerebellar ataxias constitute a heterogeneous group of disorders that result in impaired
speech, uncoordinated limb movements, and impaired balance, often ultimately resulting in …

Objective Purkinje neuron dysfunction is associated with cerebellar ataxia. In a mouse model
of spinocerebellar ataxia type 1 ( SCA 1), reduced potassium channel function contributes …

Selective neuronal vulnerability in neurodegenerative disease is poorly understood. Using
the ATXN1[82Q] model of spinocerebellar ataxia type 1 (SCA1), we explored the hypothesis …

Spinocerebellar ataxia type 3 (SCA3) is a fatal, late-onset neurodegenerative disorder
characterized by selective neuropathology in the brainstem, cerebellum, spinal cord, and …

Background A combination of central muscle relaxants, chlorzoxazone and baclofen (chlorzoxazone‐baclofen),
has been proposed for treatment of cerebellar symptoms in human …

Spinocerebellar ataxia type 3 (SCA3) is the second-most common CAG repeat disease,
caused by a glutamine-encoding expansion in the ATXN3 protein. SCA3 is characterized by …

Purkinje neuron dendritic degeneration precedes cell loss in cerebellar ataxia, but the basis
for dendritic vulnerability in ataxia remains poorly understood. Recent work has suggested …

Spinocerebellar ataxias (SCAs) are a heterogeneous group of dominantly inherited
neurodegenerative disorders affecting the cerebellum and its associated pathways. There are no …

Background Niemann–Pick disease type C is a fatal and progressive neurodegenerative
disorder characterized by the accumulation of unesterified cholesterol in late endosomes and …