Sentences with phrase «htt in»

Furthermore, postnatal inactivation of htt in brain and testis produces a progressive behavioral phenotype, neurodegeneration, and reduced survival suggesting that htt is required for neuronal function and survival (Dragatsis et al., 2000).

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.

More research is needed to further explore the role of WT htt in the adult as well as to determine what level of adult WT htt knockdown will be permissible while attempting to reduce the mutant Htt protein.

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.

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

Reduction of endogenous striatal htt using siRNA in rats expressing Htt171 - 82Q had no further detrimental effect on neuronal survival nor htt inclusion load indicating that partial inactivation of endogenous WT htt in the adult striatum (~ 50 %) had no major impact on the course of HD pathology (Drouet et al, 2009).

Not exact matches

HTT, which lays claim to the brand name «hyperloop» — a futuristic transport system that essentially involves sucking people through a vacuum tube at high speed — says it is «building the first commercial line in Abu Dhabi.»

Hyperloop Transportation Technologies (HTT) and Virgin Hyperloop One are duking it out in the Middle East.

Los Angeles - based HTT was founded in 2013, the same year SpaceX founder Elon Musk introduced the nearly 800 - mile - per - hour transportation tube concept.

In a statement, HTT chief executive officer Dirk Ahlborn said that connecting the three cities with a Hyperloop system would «incentivize collaboration and innovation within Slovakia and throughout Europe,» adding that Slovakia has been a technological leader in the automotive and energy industrieIn a statement, HTT chief executive officer Dirk Ahlborn said that connecting the three cities with a Hyperloop system would «incentivize collaboration and innovation within Slovakia and throughout Europe,» adding that Slovakia has been a technological leader in the automotive and energy industriein the automotive and energy industries.

Next week, Musk's SpaceX is hosting a capsule design competition where high school and college students will present designs in hopes of winning a $ 50,000 prize and the opportunity to work with HTT competitor Hyperloop Technologies.

In the same article, Ahlborn expressed his own frustration that Hyperloop Tech, founded after HTT, had selected such a similar company name.

Lloyd and BamBrogan also took issue with recent media reports saying that HTT was «breaking ground» on a test track in the planned Quay Valley development in California.

Hyperloop Tech's rhetoric may even show, albeit in a backhanded way, that it takes HTT seriously as a long - term competitor.

Last fall, Hyperloop Transportation Technologies (HTT), a competitor with a similar name, said it would build a five - mile test track in central California.

Earlier this March, HTT said it reached an agreement with Slovakia for a possible Hyperloop system to be built in that country.

Musk, whose interests span electric cars, solar power and space rockets, isn't interested in commercially developing the idea, so a number of companies, including HTT, have pounced on it.

Citizens in major Indian cities can see their life expectancy drop by two years amid environmental problems like air pollution, but HTT can help resolve that, Gresta said.

HTT says it was the first to take on Musk's project in 2013, and claims that the other two firms named their businesses after HTT and used its logo.

In London, at an event called Transport To The Future, HTT's chief operation officer Bibop Gresta revealed that the $ 150 million five mile test track will be built in California's Quay ValleIn London, at an event called Transport To The Future, HTT's chief operation officer Bibop Gresta revealed that the $ 150 million five mile test track will be built in California's Quay Vallein California's Quay Valley.

Concepts and renders from UCLA's Suprastudio in collaboration with HTT envision how it could pan out.

Since then, a research company called Hyperloop Transportation Technologies (HTT)-- unaffiliated with Musk or any of his other companies and ventures — is right on track to making science - fiction legit science by building an actual test track for the technology in California.

HTT plans to break ground on the project in May 2016 in Quay Valley, California.

He remains close to members of both startups currently in the lead to produce the first working Hyperloop — Hyperloop Transportation Technologies, or HTT, and Hyperloop Technologies Inc., or HTI.

HTT bought land last year to begin initial construction on the Hyperloop in Quay Valley — a proposed 75,000 resident, solar - powered concept city being built in King's County, California that intends to use HTT's test track as its main transportation system.

Built - in AR screens could also help HTT monetize in other ways — with digital advertising to a captive audience for short bursts as passengers zip from city to city.

It's about $ 20 million per mile to build HTT's version of the Hyperloop, according to Ahlborn — a fraction of the current cost of building high - speed rail in the U.S.

If HTT can prove that humans can be transported safely at such speeds, it could provoke others, including governments, to invest in making Musk's plans a reality.

It halts an intermediate step in the protein - making process by binding to genetic material known as RNA, blocking the issuing of final instructions for making the Htt protein.

Next up: a larger trial in hundreds of patients to see if lowering mutant Htt protein slows progression of the disease, then a trial in healthy people who carry the mutant HTT gene to see if antisense treatments could prevent Huntington's altogethHtt protein slows progression of the disease, then a trial in healthy people who carry the mutant HTT gene to see if antisense treatments could prevent Huntington's altogethHTT gene to see if antisense treatments could prevent Huntington's altogether.

HD is caused by a mutation in the human HTT gene that results in an abnormal expansion and misfolding of the corresponding huntingtin protein.

For example, scientists have not been able to resolve what function the HTT gene serves normally, or how its mutation creates problems in the brain.

Next we these tools to explore how mutations in the HTT protein impact on human cortical development and human neuronal and astroglial functions.

Finally we aim at identifying phenotype specific transcriptional targets of mutant HTT (mut - HTT) or HTT loss of function in relevant neuronal / glial populations.

We also investigated the effect of the adult - onset HD mutation on the role of HTT during spindle orientation in NSCs derived from HD - hESCs.

By combining SNP - targeting allele - specific silencing and gain - of - function approaches, we showed that a 46 - glutamine expansion in human HTT was sufficient for a dominant - negative effect on spindle orientation and changes in the distribution within the spindle pole and the cell cortex of dynein, p150Glued and NuMA in neural cells.

The RNAi - mediated silencing of both HTT alleles in neural stem cells derived from hESCs disrupted spindle orientation and led to the mislocalization of dynein, the p150Glued subunit of dynactin and the large nuclear mitotic apparatus (NuMA) protein.

Thus, neural derivatives of disease - specific human pluripotent stem cells constitute a relevant biological resource for exploring the impact of adult - onset HD mutations of the HTT gene on the division of neural progenitors, with potential applications in HD drug discovery targeting HTT - dynein - p150Glued complex interactions.

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

Molecular studies at PsychoGenics and other organizations have demonstrated that the excision of the neomycin resistance cassette results in a modest but significant increase in mutant HTT mRNA levels, and a trend towards an increase in soluble mHTT protein and extranuclear inclusions.

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).

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.

Knocking down the endogenous htt protein resulted in significant decrease in body weight and hyperactive behavior in the open field.

These data indicate that the disease mechanism may at least be partially driven by a loss - of - function in the htt protein.

Anticipation built in 2013 when pharmaceutical giant Roche announced a partnership with Ionis to develop the ASO drug for HD, which they call * IONIS - HTT Rx *.

These SNPs were chosen because their sequences tend to be different in the normal and mutant HTT gene: the ribbons at that point in the good and bad kites» tails tend to be a different colour, distinguishable by the drone.

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..