Boys Town Research Paper Named Editor’s Choice by The Journal of Pathology
Tuesday, September 6, 2022
A recent article by Dominic Cosgrove, Ph.D., Senior Director of the Boys Town National Research Hospital® Research Center, and his team was featured in the September issue of
The Journal of Pathology and has been selected by the Editor-in-Chief to be his Editor's Choice. The Editor's Choice award is given to the article that the editor feels is the “must read" of the issue. Dr. Cosgrove's article presents recent findings in the ongoing research of Alport syndrome, that will enable scientists to have a better understanding of the molecular mechanism of the disease.
Alport syndrome is a genetic condition characterized by kidney disease, hearing loss and eye abnormalities. It is caused by an inherited defect in type IV collagen, a structural component of basement membranes that is needed for the normal function of the kidneys, ears and eyes. This rare disease occurs in approximately 1 in 2,500 to 5,000 births.
“People with Alport syndrome experience a progressive loss of kidney function and frequently develop sensorineural hearing loss, caused by abnormalities of the inner ear," said Dr. Cosgrove. “In 1991, when I first joined Boys Town, Alport syndrome was the first and only disease that had a known genetic cause."
Dr. Cosgrove's research solves a long mystery regarding a role for collagen receptors in Alport syndrome by identifying type III collagen in capillary basement membranes of the ear and the kidney that causes progressive damage to these organs. His findings open the door to a better understanding of the mechanism of the disease. This new understanding is particularly significant as it will enable pharmaceutical companies to better treat those dealing with the disease through the establishment of a drug therapies that addresses the kidney function and hearing loss in patients with Alport syndrome.
Boys Town congratulates Dr. Cosgrove and his team on this important discovery that will lead to more effective treatment for those suffering hearing loss due to Alport syndrome.