Sentences with phrase «htt mice»

Farrar AM, Murphy CA, Paterson NE, Oakeshott S, He D, Alosio W, McConnell K, Menalled LB, Ramboz S, Park LC, Howland D, Brunner D. Cognitive deficits in transgenic and knock - in HTT mice parallel those in Huntington's disease..

Not exact matches

HTT modulates mitotic spindle orientation and cell fate in mouse cortical progenitors from the ventricular zone.

Expression of mutant Htt appeared to alter the firing rate of STN neurons, as firing rates in HET and HOM mice were lower than their WT littermate.

A 50 % reduction in HDAC4 restored these and other electrophysiological changes in both the R6 / 2 model, a transgenic over-expresser of Exon 1 HTT with an expanded polyglutamine repeat, and heterozygous Q175 knock - in mice (Q175 + / --RRB-, which carry one normal and one mutant HTT allele with an expanded repeat of ~ 190 polyglutamines, in addition to reversing behavioral alterations in R6 / 2 mice (Mielcarek et al, 2013; PLOS Biology, in press).

While Htt null KO mice do not survive to birth (Duyao et al., 1995; Nasir et al., 1995; Zeitlin et al., 1995), mice expressing a 50 % reduction of endogenous htt levels reveal cognitive and motor deficits (Nasir et al., 1995) and significant decreases in the number of neurons in globus pallidus and subthalamic nucleus (O'Kusky et al., 199Htt null KO mice do not survive to birth (Duyao et al., 1995; Nasir et al., 1995; Zeitlin et al., 1995), mice expressing a 50 % reduction of endogenous htt levels reveal cognitive and motor deficits (Nasir et al., 1995) and significant decreases in the number of neurons in globus pallidus and subthalamic nucleus (O'Kusky et al., 199htt levels reveal cognitive and motor deficits (Nasir et al., 1995) and significant decreases in the number of neurons in globus pallidus and subthalamic nucleus (O'Kusky et al., 1999).

Furthermore, postnatal inactivation of htt in brain and testes also produces a progressive behavioral phenotype in mice along with neurodegeneration and reduced survival.

In order to better understand the liabilities associated with reducing normal htt in adult mice, we evaluated the effects of a robust knock down of WT htt both in CNS and periphery in a conditional htt knock - down (KD) model induced by doxycycline.

Reduction of htt to 50 % endogenous levels produces viable mice with no overt phenotypes (Duyao et al., 1995; Zeitlin et al., 1995) however more detailed evaluation has revealed deficits in cognition and motor behavior (Nasir et al., 1995) raising the possibility that lowered htt starting in development may have adverse effects in the mouse.

Our data indicates that human htt ameliorates the cognitive deficits observed in the KD mice.

The huntingtin (htt) protein has been found to play an essential role during embryogenesis, since knockout Hdh mice do not survive gestation (Duyao et al., 1995; Nasir et al., 1995; Zeitlin et al., 1995).

We present here the first comprehensive behavioral analysis of the effects of a conditional knock - down (starting at 4.5 weeks of age) of WT endogenous htt in adult mice in the absence of any potential mutant gain - of - function.

The BAC (Bacterial Artificial Chromosome) mouse model of Huntington's disease expresses the full length human htt transgene and has been well - characterized for its progressively impaired motor function.

In addition, HDAC4 reduction delayed huntingtin (Htt) aggregation in CNS tissue from both the R6 / 2 and HdhQ150 full length KI mouse model of HD [1].