Neurodegeneration: a case of arrested development?

Cell. 2006 Nov 17;127(4):669-71. doi: 10.1016/j.cell.2006.11.010.

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.

Publication types

  • Comment

MeSH terms

  • Animals
  • Ataxin-1
  • Ataxins
  • Down-Regulation / genetics
  • Humans
  • Mice
  • Mice, Neurologic Mutants
  • Nerve Degeneration / metabolism*
  • Nerve Tissue Proteins / deficiency
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism
  • Nuclear Proteins / deficiency
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • Nuclear Receptor Subfamily 1, Group F, Member 1
  • Purkinje Cells / cytology
  • Purkinje Cells / pathology
  • Receptors, Cytoplasmic and Nuclear / metabolism
  • Trans-Activators / metabolism

Substances

  • ATXN1 protein, human
  • Ataxin-1
  • Ataxins
  • Atxn1 protein, mouse
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • Nuclear Receptor Subfamily 1, Group F, Member 1
  • RORA protein, human
  • Receptors, Cytoplasmic and Nuclear
  • Trans-Activators