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Maze-solving dachshunds help treat neurological disorders

Posted May 26, 2012
By Grace Olinger

Every year in April, the University of Missouri hosts Life Sciences Week, an event where scientists come together at the Bond Life Science Center to present their research in many forms, including poster presentations and seminars.

Among the presenters was Jacob Taylor, a junior biology major conducting research on a neurological genetic disorder called neuronal ceroid lipofuscinosis (NCL).

NCL is a group of rare, inherited diseases that affect humans and canines. There are three types: adult, juvenile and late infantile. All three types cause mental retardation, loss of vision and lack of motor control. The juvenile form, also known as Batten disease, is the most well-known type. It is a devastating disease in which the child might have fewer than 10 years to live.

 

[media-credit id=59 align="alignleft" width="477"][/media-credit]Taylor began his research on these diseases in his sophomore year in the lab of Martin Katz, professor of ophthalmology.

 

“When I saw that one of the openings listed ‘playing with puppies’ as a job perk, I was hooked, and have been working there ever since,” Taylor said.

Taylor and others in Katz’s lab study these diseases using long-haired miniature dachshunds as an animal model, but Katz says the research benefits both dogs and people.

Taylor agrees: “I hope that my research, and the data I am able to acquire, can be used to provide enough support to take our treatments to human trials. This way, I could have in some way contributed to curing Batten’s disease in children, improving and lengthening their lives.”

Taylor has been studying the effects late-infantile form of NCL and a possible treatment in the form of an enzyme known as TPP1, which is responsible for breaking down the protein build-up in the brain and is not functional in those affected with NCL.

He trained dogs to solve a maze so he could devise a way to observe the degeneration of cognitive function over time in dogs afflicted with NCL. Such a test allows Taylor to observe and measure any cognitive improvement after treatment with TPP1. “There is no perfect way of measuring the cognitive function of dogs, so we had to be creative, and the maze proved to be a good indicator,” Taylor said.

His work showed that the dogs with NCL will make an increasing number of mistakes as the disease progresses. Many of these untreated dogs did not live past nine months. However, after receiving treatment, the dogs in the treatment group were still alive 15 months into the study when Taylor collected the last set of data.

These data show that treatment with TTP1 is effective in the recovery of cognitive function and that it could also lengthen the dogs’ lives.

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C3 Transforming Life Sciences Through Collaboration X Computation X Communication University of Missouri HHMI