2003
DOI: 10.1093/hmg/ddg075
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Transgenic rat model of Huntington's disease

Abstract: Huntington's disease (HD) is a late manifesting neurodegenerative disorder in humans caused by an expansion of a CAG trinucleotide repeat of more than 39 units in a gene of unknown function. Several mouse models have been reported which show rapid progression of a phenotype leading to death within 3-5 months (transgenic models) resembling the rare juvenile course of HD (Westphal variant) or which do not present with any symptoms (knock-in mice). Owing to the small size of the brain, mice are not suitable for r… Show more

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Cited by 283 publications
(65 citation statements)
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“…After 4 days of training (trial duration of 120 s twice daily with an intertrial interval of 90 min) at a constant speed of 14 rpm, animals were subjected to the apparatus for 4 days, and the rotation speed was constantly increased (4-40 rpm) over a 5-min time period. The latency to fall off the rod and the actual rotation speed were recorded (25). Beam walking test.…”
Section: Methodsmentioning
confidence: 99%
“…After 4 days of training (trial duration of 120 s twice daily with an intertrial interval of 90 min) at a constant speed of 14 rpm, animals were subjected to the apparatus for 4 days, and the rotation speed was constantly increased (4-40 rpm) over a 5-min time period. The latency to fall off the rod and the actual rotation speed were recorded (25). Beam walking test.…”
Section: Methodsmentioning
confidence: 99%
“…Differences between rats and mice have also been described in relation to modelling HD, with some studies suggesting that the rat provides a more faithful model of the human disease than the mouse (von Horsten et al, 2003), especially in terms of progression, which seems much faster in mice than in rats (and humans). However, this conclusion is mainly based on a comparison with the R6/2 mouse model, which shows a very fast-progressing and severe phenotype (Mangiarini et al, 1996), even compared to other mouse models.…”
Section: Rats and Mice As Models For Human Neurological And Psychiatrmentioning
confidence: 99%
“…For this purpose, we investigated the mitochondrial function in a newly generated transgenic HD rat strain with 51 glutamine repeats (htt 51Q ) (20). In contrast to htt Q150 R6/2 mice, a model of the juvenile form of HD (21), the htt 51Q rat strain exhibits specifically an adult-related onset of the neurological HD phenotype (20).…”
Section: Huntington Disease (Hd)mentioning
confidence: 99%