Voor het Angelman Syndroom

pdf Loss of dopaminergic neurons and resulting behavioral deficits in mouse model of Angelman syndrome Populair

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Mulherkar SA 2010 Neurobiol Dis e-pub.pdf

E6 associated protein is an E3 ubiquitin ligase encoded by the gene Ube3a. Deletion orloss of function of the maternally inherited allele of Ube3a leads to Angelman syndrome.In the present study, we show that maternal loss of Ube3a (Ube3am-/p+) in the mousemodel leads to motor deficits that could be attributed to the dysfunction of thenigrostriatal pathway. The number of tyrosine hydroxylase positive neurons in thesubstantia nigra was significantly reduced in Ube3am-/p+ mice as compared to the wildtype counterparts. The Ube3am-/p+ mice performed poorly in behavioral paradigmssensitive to nigrostriatal dysfunction. Even though the tyrosine hydroxylase staining wasapparently same in the striatum of both genotypes, the presynaptic and postsynapticproteins were significantly reduced in Ube3am-/p+ mice. These findings suggest that theabnormality in the nigrostriatal pathway along with the cerebellum produces the observedmotor dysfunctions in Ube3am-/p+ mice.

Shalaka A. Mulherkar and Nihar Ranjan Jana

Cellular and Molecular Neuroscience Laboratory, National Brain Research Centre,Manesar, Gurgaon - 122 050, India

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