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Changes in GAD67 mRNA expression evidenced by in situ hybridization in the brain of R6/2 transgenic mice by I. Gourfinkel-An; K. Parain; A. Hartmann; L. Mangiarini; A. Brice; G. Bates; E. C. Hirsch is a Neuroscience article available to read on EtoBox.

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## Abstract Huntington's disease is an autosomal dominant disorder with degeneration of medium size striatal neurones. As the disease evolves, other neuronal populations are also progressively affected. A transgenic mouse model of the disease (R6/2) that expresses exon 1 of the human Huntington gene with approximately 150 CAG repeats has been developed, but GABA concentrations are reported to be normal in the striatum of these animals. In the present study, we analysed the status of GABAergic systems by means of glutamic acid decarboxylase (GAD)~67~ mRNA __in situ__ hybridization in the brain of R6/2 transgenic mice and wild‐type littermates. We show that GAD~67~ expression is normal in the striatum, cerebellum and septum but decreased in the frontal cortex, parietal cortex, globus pallidus, entopeduncular nucleus and substantia nigra pars reticulata of R6/2 mice. These data, which may, in part, account for the behavioural changes seen in these animals, indicate that at 12.5 weeks of age the pathological features seen in the mice differ from those seen in humans with Huntington's disease.

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Author

I. Gourfinkel-An; K. Parain; A. Hartmann; L. Mangiarini; A. Brice; G. Bates; E. C. Hirsch

Publisher

John Wiley and Sons; Wiley (Blackwell Publishing); Blackwell Publishing Inc.; Wiley (ISSN 0022-3042)

Published

2003

Language

EN

Field

Neuroscience (Life Sciences)