The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
A role for apoptotic mediators in neurodegenerative disease has long been considered, but the identity of the putative effectors and the basis of their actions in neurodegeneration remain ill-defined. Huntington's disease (HD) is a polyglutamine disorder whose pathogenesis likely involves mitochondrial dysfunction. In this issue of Neuron, Bae et al. present data implicating p53 in HD pathogenesis. This intriguing study may thus provide a link between nuclear transcription dysregulation and mitochondrial abnormalities in HD.
La Spada
Albert R
AR
Department of Laboratory Medicine, University of Washington, Seattle, WA 98195, USA.
Morrison
Richard S
RS
D000818:Animals; D016158:Genes, p53 / Q000502:physiology; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics* / Q000473:pathology*; D051379:Mice; D008822:Mice, Transgenic; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000502:physiology*; D009687:Nuclear Proteins / Q000235:genetics* / Q000502:physiology*; D016159:Tumor Suppressor Protein p53 / Q000502:physiology*
C086055:HTT protein, human; C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D016159:Tumor Suppressor Protein p53