One Family's Search to Explain a Fatal Neurological Disorder
With their help, researchers are advancing the 70-year effort to understand—and treat—heredetary ataxia.
When Henry Schut was a boy in the early 1900s, he watched his father slowly die from a mysterious ailment, progressively losing the ability to walk, to speak, and, eventually, to breath. As Henry and his siblings took over running the family farm, and, as they grew older, they watched as more family members succumbed to the disease. Henry's brother John, compelled by the suffering in his family due to what they eventually found out was called hereditary ataxia, entered medical school with the quest to research and hopefully prevent future suffering in his family. John knew he was at risk for the dominant genetic disorder, and eventually, he, too, succumbed to the disease. His research legacy passed on to his nephew Larry, Henry's son, who was finishing medical school. Because of the research efforts of the Schuts and others, the genetic and neurological underpinnings of the disease have been revealed over the past few decades, discoveries advanced with each new development in molecular techniques. As the complexities of the disease have come to light, the medical community approaches the possibility for a cure for the hereditary ataxia that ran in the Schut family, called spinocerebellar ataxia type 1, which is one of several ataxic disorders that plague 150,000 people in the United States alone.
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