Unraveling the contribution of oligodendrocytes to neurodegeneration: a new look at Wolfram's disease. KU Leuven
Wolfram syndrome is a rare hereditary disease, causing diabetes, blindness, deafness and other neurological problems in infants and young adults, and results in death around the age of 30 years. Wolfram patients carry recessive mutations in the WFS1 gene, resulting in loss of function of the WFS1 protein. Loss of WFS1 has been shown to result in ER stress, dysregulated Ca2+ homeostasis and mitochondrial dysfunction. Remarkably, in the brains ...