Neurodegeneration: a case of arrested development?
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.
La Spada
Albert R
AR
Department of Laboratory Medicine, University of Washington, Seattle, WA 98195, USA. laspada@u.washington.edu
D000818:Animals; D000067696:Ataxin-1; D000067528:Ataxins; D015536:Down-Regulation / Q000235:genetics; D006801:Humans; D051379:Mice; D008818:Mice, Neurologic Mutants; D009410:Nerve Degeneration / Q000378:metabolism*; D009419:Nerve Tissue Proteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism; D009687:Nuclear Proteins / Q000172:deficiency / Q000235:genetics / Q000378:metabolism; D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; D011689:Purkinje Cells / Q000166:cytology / Q000473:pathology; D018160:Receptors, Cytoplasmic and Nuclear / Q000378:metabolism; D015534:Trans-Activators / Q000378:metabolism
C000593749:ATXN1 protein, human; D000067696:Ataxin-1; D000067528:Ataxins; C000593750:Atxn1 protein, mouse; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; C539738:RORA protein, human; D018160:Receptors, Cytoplasmic and Nuclear; D015534:Trans-Activators