Neurodegeneration: a case of arrested development?

La Spada, Albert R · Cell · 2006

editorial · Level V

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Abstract

The neurodegenerative disease Spinocerebellar ataxia type 1 (SCA1) is a polyglutamine expansion disorder characterized by the death of Purkinje neurons in the brain. In this issue, Serra et al. (2006) implicate the impaired function of the orphan nuclear receptor RORalpha in SCA1 pathogenesis. Their intriguing results suggest that derailing a transcription program during embryonic development may render adult neurons more susceptible to toxic insults.

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