Scientists Discover a Better Batten Disease Model In Primates

According to a report by Science Daily, researchers recently discovered a disease in monkeys which imitates human Batten disease. Establishing the closest analog to the condition in humans, the version found in monkeys holds great potential for the development of new treatments and therapies. Keep reading to learn more, or follow the original story here for more information.

What is Batten Disease?

Batten disease is an inherited condition affecting the nervous system. Typically, Batten disease develops during childhood, and is ultimately fatal. Batten disease is a lysosomal disorder, affecting the way the body processes lipopigments. Lipopigments are made up of fats and proteins. Their accumulation leads to the destruction of neurons in the brain, retina, and central nervous system (CNS).

Common symptoms of Batten disease include seizures, visual impairment, changes in personality, dementia, and loss of motor and communication skills. Batten disease may also be referred to as neuronal ceroid lipofuscinosis, or NCL. Treatments currently focus on patient comfort, and slowing the progression of symptoms since no cure has been discovered.

Click here to learn more about Batten disease.

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Until now, most treatments for Batten disease have been tested on mice. These mice had to be genetically manipulated to create a condition similar to Batten disease. While this kind of test model is preferable to no test model, mice are not always perfect representatives. They do not always have the same response to therapies as humans do, nor do the target diseases always behave the same way as they do in humans. Further more, the disease must be introduced to the mouse population. This carries both scientific and ethical concerns.

Finding a naturally occurring disease which is more similar to Batten disease is a great step then.

The discovery was made in what researchers are calling a collaborative effort. Researchers members from the Oregon National Primate Research Center (ONPRC) made the initial discovery. The team covers a broad spectrum of disciplines, and their full report can be found in the journal Neurobiology of Disease.

Fast Track

OHSU scientists didn’t, however, stop at simply observing this phenomenon. The study’s co-author says they are very aware of what’s at stake with this kind of discovery. The combination of a naturally occurring model in a non-human primate and modern gene therapy technology will likely accelerate scientists towards better treatments for Batten disease.

Scientists at OHSU are, in fact, in the process of developing such therapies at the time of this article. Researchers studying gene therapies are investigating similar degenerative diseases in non-human primates and the results may provide useful crossover.

The discovery of this primate model of Batten disease opens up many possibilities for the study of biomarkers as well. These indicators may help scientists better understand and track the progression of Batten disease in humans. Scientists at the ONPRC describe their goals as slowing and stopping Batten disease. They aim to do this as soon as possible with the information from the new model.

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