Toward cell specificity in SCA1

Neuron. 2002 May 30;34(5):669-70. doi: 10.1016/s0896-6273(02)00715-8.

Abstract

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.

Publication types

  • Review
  • Comment

MeSH terms

  • Ataxin-1
  • Ataxins
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism
  • Cell Death / genetics
  • DNA-Binding Proteins
  • Humans
  • Huntingtin Protein
  • Huntington Disease / genetics*
  • Huntington Disease / metabolism
  • Huntington Disease / physiopathology
  • Nerve Tissue Proteins / genetics*
  • Nerve Tissue Proteins / metabolism
  • Nuclear Proteins / genetics*
  • Nuclear Proteins / metabolism
  • Peptides / genetics
  • Peptides / metabolism
  • Spinocerebellar Ataxias / genetics*
  • Spinocerebellar Ataxias / metabolism
  • Spinocerebellar Ataxias / physiopathology
  • Transcription Factors / genetics
  • Trinucleotide Repeat Expansion / genetics

Substances

  • ATXN1 protein, human
  • Ataxin-1
  • Ataxins
  • Carrier Proteins
  • DNA-Binding Proteins
  • HTT protein, human
  • Huntingtin Protein
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • PQBP1 protein, human
  • Peptides
  • Transcription Factors
  • polyglutamine