Scientists from Japan’s Kumamoto University have discovered a protein that puts the brakes on the progression of Alport syndrome, a rare genetic kidney disease that can affect children and young people and often leads to kidney failure. The protein, known as p53, is a tumor suppressor, already targeted by experimental cancer drugs. Using a mouse model of Alport syndrome, the researchers found that p53 contributes to the health and formation of podocyte cells, which make up the kidney filtration barrier. They also noted that p53 activity drops as Alport syndrome progresses. They believe that therapies to increase p53 activity could prevent kidney failure. Currently, with the most common X-linked form of Alport syndrome, about 50% of males will develop kidney failure by the age of 25 and 90% will progress to kidney failure before age 40.
Protein That Limits the Severity of Genetic Kidney Disease Found, EurekAlert, February 9, 2016
Podocyte p53 Limits the Severity of Experimental Alport Syndrome, Journal of the American Society of Nephrology, January 2016