The Lurcher mouse

Fresh insights from an old mutant

Michael W. Vogel, Jean Caston, Michisuke Yuzaki, Jean Mariani

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

The Lurcher mouse was first discovered in 1954 as a spontaneously occurring autosomal dominant mutation that caused the degeneration of virtually all cerebellar Purkinje cells and most olivary neurons and granule cells. More recent molecular studies revealed that Lurcher is a gain of function mutation in the δ2 glutamate receptor (GluRδ2) that converts an alanine to threonine in the highly conserved third hydrophobic segment of GluRδ2. The mutation converts the receptor into a constitutively leaky cation channel. The GluRδ2 receptor is predominantly expressed in cerebellar Purkinje cells and in the heterozygous Lurcher mutant (+/Lc). Purkinje cells die due to the mutation in the GluRδ2 receptor, while olivary neurons and granule cells degenerate due to the loss of their Purkinje cell targets. The purpose of the review is to provide highlights from 5 decades of research on the Lurcher mutant that have provided insights into the developmental mechanisms that regulate cell number during development, cerebellar pattern formation, cerebellar physiology, and the role of the cerebellum in CNS function.

Original languageEnglish
Pages (from-to)4-18
Number of pages15
JournalBrain Research
Volume1140
Issue number1
DOIs
Publication statusPublished - 2007 Apr 6

Fingerprint

Neurologic Mutant Mice
Purkinje Cells
Mutation
Neurons
Glutamate Receptors
Threonine
Alanine
Cerebellum
Cations
Cell Count
Research
AMPA 2 glutamate receptor ionotropic

Keywords

  • Cerebellum
  • Glutamate receptor
  • Granule cell
  • Mouse mutant
  • Neurodegeneration
  • Purkinje cell

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology
  • Developmental Biology
  • Molecular Biology

Cite this

The Lurcher mouse : Fresh insights from an old mutant. / Vogel, Michael W.; Caston, Jean; Yuzaki, Michisuke; Mariani, Jean.

In: Brain Research, Vol. 1140, No. 1, 06.04.2007, p. 4-18.

Research output: Contribution to journalArticle

Vogel, Michael W. ; Caston, Jean ; Yuzaki, Michisuke ; Mariani, Jean. / The Lurcher mouse : Fresh insights from an old mutant. In: Brain Research. 2007 ; Vol. 1140, No. 1. pp. 4-18.
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