Rescue of cerebellar granule cells from death in weaver NR1 double mutants

J Neurosci. 1999 Sep 15;19(18):7991-8. doi: 10.1523/JNEUROSCI.19-18-07991.1999.

Abstract

The weaver mutation results in the extensive death of midline cerebellar granule cells. The mutation consists of a single base pair substitution of the gene encoding the G-protein-activated inwardly rectifying potassium channel protein, GIRK2. The functional consequences of this mutation are still in dispute. In this study we demonstrate the in vivo and in vitro rescue of weaver granule cells when NR1 NMDA subunits are eliminated in weaver NR1 double mutants. This rescue of weaver granule cells provides evidence that wvGIRK2 alone is not sufficient to cause granule cell death.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Aging
  • Amino Acid Substitution
  • Animals
  • Animals, Newborn
  • Ataxia / genetics
  • Ataxia / physiopathology
  • Cell Death
  • Cell Survival
  • Cerebellum / abnormalities
  • Cerebellum / cytology*
  • Cerebellum / physiology*
  • G Protein-Coupled Inwardly-Rectifying Potassium Channels
  • Genotype
  • Male
  • Mice
  • Mice, Knockout
  • Mice, Neurologic Mutants
  • Neurons / cytology
  • Neurons / physiology*
  • Polymerase Chain Reaction
  • Potassium Channels / analysis
  • Potassium Channels / genetics
  • Potassium Channels / physiology*
  • Potassium Channels, Inwardly Rectifying*
  • Receptors, N-Methyl-D-Aspartate / deficiency
  • Receptors, N-Methyl-D-Aspartate / genetics
  • Receptors, N-Methyl-D-Aspartate / physiology*
  • Reference Values

Substances

  • G Protein-Coupled Inwardly-Rectifying Potassium Channels
  • Potassium Channels
  • Potassium Channels, Inwardly Rectifying
  • Receptors, N-Methyl-D-Aspartate