Toward cell specificity in SCA1.
Transcriptional dysregulation appears as an emerging and unifying pathogenic mechanism in polyQ neurodegenerative disorders such as Spinocerebellar ataxias and Huntington's disease. It is unclear how cell death specificity occurs in these diseases. In this issue of Neuron, link polymerase II, a general component of the transcriptional machinery, to PQBP-1, a cerebellar enriched protein, thus providing insight into the selectivity of neuronal death in SCA1.
Humbert
Sandrine
S
UMR 146 CNRS, Institut Curie, Bldg. 110, Centre Universitaire, 91405 Orsay Cedex, France.
Saudou
Frédéric
F
D000067696:Ataxin-1; D000067528:Ataxins; D002352:Carrier Proteins / Q000235:genetics / Q000378:metabolism; D016923:Cell Death / Q000235:genetics; D004268:DNA-Binding Proteins; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000378:metabolism; D009687:Nuclear Proteins / Q000235:genetics* / Q000378:metabolism; D010455:Peptides / Q000235:genetics / Q000378:metabolism; D020754:Spinocerebellar Ataxias / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D014157:Transcription Factors / Q000235:genetics; D019680:Trinucleotide Repeat Expansion / Q000235:genetics
C000593749:ATXN1 protein, human; D000067696:Ataxin-1; D000067528:Ataxins; D002352:Carrier Proteins; D004268:DNA-Binding Proteins; C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; C119246:PQBP1 protein, human; D010455:Peptides; D014157:Transcription Factors; C097188:polyglutamine