Differential changes in striatal projection neurons in R6/2 transgenic mice for Huntington's disease

Z. Sun, N. Del Mar, C. Meade, D. Goldowitz, Anton Reiner

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

In early adult-onset Huntington's disease (HD), enkephalinergic striatopallidal projection neurons show preferential loss, reduced preproenkephalin (PPE) expression in surviving striatopallidal neurons, and loss of fibers in their projection target area. We examined PPE and PPT (preprotachykinin) gene expression in striatal projection neurons and in striatal projection fibers immunoreactive for the PPE product enkephalin (ENK) and the PPT product substance P (SP) in a transgenic HD model, the R6/2 mouse, to see if changes occur in these neuron types similar to those seen in early adult-onset HD. The results show that PPE mRNA level, the number of striatal neurons expressing PPE, and the staining intensity of fibers immunoreactive for ENK in the pallidum were all decreased. By contrast, the SP-containing striatal projection systems to the pallidum and substantia nigra were relatively normal in R6/2 mice. The selective reduction in striatal PPE in R6/2 mice is reminiscent of adult-onset HD, but the preservation of the striatonigral projection system is not. Thus, R6/2 mice do not strictly mimic adult-onset HD in their striatal pathology.

Original languageEnglish (US)
Pages (from-to)369-385
Number of pages17
JournalNeurobiology of Disease
Volume11
Issue number3
DOIs
StatePublished - Jan 1 2002

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Corpus Striatum
Huntington Disease
Transgenic Mice
Neurons
Globus Pallidus
Enkephalins
Substance P
Substantia Nigra
preproenkephalin
Pathology
Staining and Labeling
Gene Expression
Messenger RNA

All Science Journal Classification (ASJC) codes

  • Neurology

Cite this

Differential changes in striatal projection neurons in R6/2 transgenic mice for Huntington's disease. / Sun, Z.; Del Mar, N.; Meade, C.; Goldowitz, D.; Reiner, Anton.

In: Neurobiology of Disease, Vol. 11, No. 3, 01.01.2002, p. 369-385.

Research output: Contribution to journalArticle

Sun, Z. ; Del Mar, N. ; Meade, C. ; Goldowitz, D. ; Reiner, Anton. / Differential changes in striatal projection neurons in R6/2 transgenic mice for Huntington's disease. In: Neurobiology of Disease. 2002 ; Vol. 11, No. 3. pp. 369-385.
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