Bad fats in the brain: Loss of fat-modifying enzyme is the cause of a childhood brain disease
Original Scientific Article, D.Pant et al: 
Written By: Kathleen Cunningham
The Gist of It:
In your brain and spinal cord, neurons have a fatty covering that protects them, similar to the plastic coating around your cell phone charge cord. This covering is made of myelin. Myelin is made by cells in your brain called oligodendrocytes and it has many roles, including helping to maintain the proper electrical signals in the neurons in your brain. However, in patients with leukodystrophies (LD), a group of rare disorders that affect the nervous system, myelin is not made or broken down abnormally Patients with LD develop the disorder in infancy and can have severe symptoms, such as poor motor function, involuntary muscle contractions, seizures, and death.
Using genetic sequencing, Pant and colleagues found a change in the gene DEGS1 that caused LD in 19 patients from 13 different families.
Zebrafish were used to test whether DEGS1 mutations identified in leukodystrophy patients can cause features similar to disease in humans and to test possible treatments.
