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University of Missouri

The Truth About Katz and Dogs

Dachshunds help doctors treat Batten disease.

dachshund

Longhaired dachshunds are a good animal model for Mizzou researchers studying Batten disease, an illness that also affects humans. ©shutterstock.com

Batten disease is a rare, degenerative and always fatal neurological disorder that affects up to four children per 100,000 U.S. births. The symptoms, which begin at various stages of childhood development, include seizures, mental impairment, blindness and paralysis. No cure exists. Yet.

Some dachshunds also carry Batten disease, and a treatment developed by Martin Katz, a professor of ophthalmology with dual appointments in the School of Medicine and the College of Veterinary Medicine, is sparking hope for man and beast.

Batten disease occurs when an important enzyme in neural cells is missing. This enzyme helps cells break down and eliminate waste proteins. Without it, waste accumulates and cells cannot function properly.

Katz’s therapy injects the enzyme into the afflicted canine’s neural system, restoring some nervous system functions.

“The treatment dramatically slowed all the neurological signs [of damage] in the dogs,” Katz says. “The life spans were significantly extended. It didn’t cure the disease, but it slowed down progression.”

Katz’s results in dogs have prompted early human studies in Germany and the United Kingdom. His goal is to develop a treatment wherein the patient — dog or human — can permanently produce enough of the enzyme to cure the disease.