Deactivation of TBP contributes to SCA17 pathogenesis

Age-Dependent Decrease in Chaperone Activity Impairs MANF Expression, Leading to Purkinje Cell Degeneration in Inducible SCA17 Mice

(2014) Su Yang et al.   NEURON

Role of the CCAAT-Binding Protein NFY in SCA17 Pathogenesis

(2012) Li-Ching Lee et al.   PLoS One

The Relationship between Aggregation and Toxicity of Polyglutamine-Containing Ataxin-3 in the Intracellular Environment of Escherichia coli

(2012) Gaetano Invernizzi et al.   PLoS One

Neuronal expression of TATA box-binding protein containing expanded polyglutamine in knock-in mice reduces chaperone protein response by impairing the function of nuclear factor-Y transcription factor

(2011) Shanshan Huang et al.   BRAIN

A Drosophila model of the neurodegenerative disease SCA17 reveals a role of RBP-J/Su(H) in modulating the pathological outcome

(2011) Jie Ren et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Dysfunction and Oxidative Stress Contribute to the Pathogenesis of Spinocerebellar Ataxia Type 12 (SCA12)

(2011) Yu-Chun Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pathogenic VCP/TER94 Alleles Are Dominant Actives and Contribute to Neurodegeneration by Altering Cellular ATP Level in a Drosophila IBMPFD Model

(2011) Ya-Chu Chang et al.   PLoS Genetics

SCA17 repeat expansion: Mildly expanded CAG/CAA repeat alleles in neurological disorders and the functional implications

(2009) Chiung-Mei Chen et al.   CLINICA CHIMICA ACTA

Liquid chemiluminescent DNA pull-down assay to measure nuclear receptor-DNA binding in solution

(2008) Toshiharu Iwasaki et al.   BIOTECHNIQUES

Spinocerebellar ataxia 17 (SCA17) and Huntington’s disease-like 4 (HDL4)

(2008) Giovanni Stevanin et al.   CEREBELLUM

Altered expression of HSPA5, HSPA8 and PARK7 in spinocerebellar ataxia type 17 identified by 2-dimensional fluorescence difference in gel electrophoresis

(2008) Li-Ching Lee et al.   CLINICA CHIMICA ACTA

Polyglutamine Expansion Reduces the Association of TATA-binding Protein with DNA and Induces DNA Binding-independent Neurotoxicity

(2008) Meyer J. Friedman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntington's disease phenocopies are clinically and genetically heterogeneous

(2008) Edward J. Wild et al.   MOVEMENT DISORDERS