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'Disease in a Dish' Helps Experts Fight Huntingtons

Sep 05, 2014 03:20 PM EDT

Huntington's disease is a difficult-to-study and very sneaky genetic condition that always results in death. Now researchers believe they have managed to create a model from primate cells that allows them to study the effects of the disease in accelerated time, and all in  a Petri dish.

They are calling it "disease in a dish" and it's a useful tool that is not to be taken lightly. The effects of many disease have long been studied in labs under microscope, but as Huntington's disease (HD) is a genetic condition, it is more difficult to observe.

In humans, Huntington's is a very scary condition. It can be passed over via near-dominant genes from parent to child, and usually only expresses itself when a person is past the age of 35 - long after they likely already had children.

According to the World Health Organization, there is a 50/50 chance of inheriting the fatal gene from a parent, and it eventually leads to complete neurodegenerative breakdown, where the afflicted patient will eventually no longer to walk, talk, or reason, effectively.

Waiting 35 years or more to study the effects of this disease can severely slow scientific understanding of it, so back in 2008 researcher Anthony Chan of the Yerkes National Primate Research Center at Emory University helped his colleagues create a primate model for lab testing that experienced the onset of HD only two years into development.

Now, taking things a step forward once more, the same researcher and his colleagues have discovered how to make  nerve cells in a Petri dish come down with the disorder.

A study recently published in the journal Stem Cell Reports, details how Chan and his team used induced pluripotent stem cells (iPS) to create neural progenitor cells affected with HD for study. Like most iPS, these cells were derived from the skin cells of an organisms - in this case the transgenic monkey models. This allows the progression of HD to be closely analyzed "in a dish" and in real-time with very little wait.

"A highlight of our model is that our progenitor cells and neurons developed cellular features of HD such as intranuclear inclusions of mutant Huntingtin protein, which most of the currently available cell models do not present," Chan added in a recent release. "We could use these features as a readout for therapy using drugs or a genetic manipulation."

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