This is the regulation of basic cellular processes that happen to rid the body of harmful waste. Removal of toxic materials from the brain requires efficient lysosomes that assist in clearing deposits from anywhere in the body, and the transcription factor EB (TFEB) that is responsible for the production of functional lysosomes. “We have found that oral gemfibrozil successfully reduces inflammation in the brain, decreases brain accumulation of toxic autofluorescent pigment deposits and improves locomotor activities in mice that are missing the CLN3 gene,” Pahan said. Death usually occurs in the 20s, depending on the speed of disease progression. Life expectancy varies depending on the type or variation. Juvenile Batten disease is a life-limiting disease. Reduces brain inflammationĬhildren with Batten disease eventually become blind, bedridden and demented. “Finding an effective drug to protect the brain and stop the progression of Batten is an important area of research,” said Kalipada Pahan, PhD, the Floyd A. Currently, no drug is available to slow or halt the disease progression. Batten disease is inherited in an autosomal recessive pattern in the loss of a specific gene, CLN3, which provides instructions for making a protein that is found in tissues throughout the body. It occurs in an estimated two to four out of every 100,000 births in the United States. Juvenile Batten disease is a rare but devastating neurodegenerative disorder that begins in childhood, causing loss of vision, seizures, and progressive neurological degeneration, which is a loss of structure and function of the brain. Autofluorescent pigment deposits are made up of lipid-protein fragments that increase in the brain over time and suffocate healthy cells by depriving them of oxygen, which leads to neuronal death. Researchers found that gemfibrozil, which has the brand name Lopid, assists with clearing the brain of autofluorescent pigment deposits, which is a hallmark of the disease. Results from the study were recently published in the Journal of Neuroscience. The FDA-approved, lipid-lowering medication gemfibrozil may prevent the progression of juvenile Batten disease, according to researchers at RUSH.
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