Browsing by Author "La Spada, Albert R"

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Motor neuron degeneration in spinal and Bulbar Muscular Atrophy is a skeletal muscle-driven process: Relevance to therapy development and implications for related motor neuron diseases.

Cortes, Constanza J; La Spada, Albert R (Rare Dis, 2014)

Non-cell autonomous degeneration has arisen as an important mechanism in neurodegenerative disorders. Using a novel line of BAC androgen receptor (AR) transgenic mice with a floxed transgene (BAC fxAR121), we uncovered a ...
PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically.

Dickey, Audrey S; Pineda, Victor V; Tsunemi, Taiji; Liu, Patrick P; Miranda, Helen C; Gilmore-Hall, Stephen K; Lomas, Nicole; ... (23 authors) (Nat Med, 2016-01)

Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the huntingtin (HTT) gene, which encodes a polyglutamine tract in the HTT protein. We found that peroxisome ...