Scientists Show Simple Sugar Stops Neurodegeneration

Scientists Show Simple Sugar Stops Neurodegeneration News Jun 21, 2018 | Original Story by Allison Mickey for Baylor College of Medicine Simple Sugar Prevents Neurodegeneration in Lysosomal Storage Disease Credit: Pixabay AddThis Sharing Buttons Share to FacebookShare to TwitterShare to LinkedInShare to Google+Share to WhatsAppShare to More A new therapeutic approach may one day delay neurodegeneration typical of a disease called mucopolysaccharidoses IIIB (MPS IIIB). Neurodegeneration in this condition results from the abnormal accumulation of essential cellular molecules called mucopolysaccharides. Looking to find alternative therapeutic strategies for this rare genetic disease, a team of researchers investigated whether enhancing the cells’ ability to clear accumulation of cellular waste would help eliminate the abnormal storage of mucopolysaccharides. They report in the journal Autophagy that the sugar trehalose increases cellular waste disposal and improves the neurological symptoms in a mouse model of the disease. “MPS IIIB is one of about 50 lysosomal storage disorders characterized by the accumulation of material inside tiny cellular sacs called lysosomes,” said corresponding author Dr. Marco Sardiello, assistant professor of molecular and human genetics and a member of the Jan and Dan Duncan Neurological Research Institute at Texas Children’s Hospital and Baylor College of Medicine. “In the case of MPS IIIB, a mutation on a gene that codes for a lysosomal enzyme that breaks down a cellular material called heparan sulfate, renders the enzyme ineffective. Consequently, the lysosome cannot do its work of degrading heparan sulfate to either discard it or recycle it, and the material accumulates.” Over the years, accumulation of heparan sulfate in lysosomes leads to degeneration of brain tissue. Although infants appear healthy at first, they slowly begin to show behavioral problems, hyperactivity, aggressiveness, sleep disturbances and loss of vision and hearing. Later in life, they become immobile and develop swallowing difficulties. Usually, they don’t live past the second decade. Current strategies being tested for the treatment of this condition in animal models include attempting to correct the enzyme deficiency by providing a fully working enzyme. However, this approach faces challenges such as having limited ability to cross the blood-brain barrier and reaching the brain areas where the enzyme is needed. https://www.technologynetworks.com/neuroscience/news/simple-sugar-prevents-neurodegeneration-in-lysosomal-storage-disease-305244?utm_campaign=Newsletter_TN_BreakingScienceNews&utm_source=hs_email&utm_medium=email&utm_content=63934286&_hsenc=p2ANqtz-_KSihquDbCd801Vm1SkdkaNyj1SxyuamoU47xrTyQFUF4XJ9lLL5YEFWc6Gj1Yp6xOUqNe7RK2aAXZfhKfT4mnN2nHmg&_hsmi=63934286