Williams-Beuren Syndrome (WBS) is a disorder that causes problems with cognitive function and development. However, the disorder can sometimes enhance auditory abilities. Why do people with WBS have better linguistic and musical ability than others? Scientists at St. Jude now have an answer.
The brain processes sound in an area called the auditory cortex. The scientists showed that the enhanced auditory ability is associated with hyperexcitable neurons in the auditory cortex.
Genetic data led the scientists to a neuropeptide receptor called VIPR1, which is reduced in the auditory cortex in WBS.
A gene that regulates VIPR1 is among the 27 deleted genes in WBS patients. The relationship between the gene and VIPR1 is responsible for the increased auditory ability in the syndrome.
“This work suggests that reducing neuronal hyperexcitability might be a general mechanism for treating WBS through targeting VIPR1,” said Stanislav Zakharenko, MD, PhD, St. Jude Developmental Neurobiology. “It also opens up new directions to learn about musical ability and how our brain differentiates sounds.”
The findings appeared in Cell.