Investigations on Genetic Neurological Disorders of The Central Nervous System Involving Primarily Myelin Metabolism and Their Animal Counterparts

Several genetic neurological diseases involve primarily myelin or the myelin supporting cell, i.e. the oligodendrocyte in the central nervous system, or the Schwann cell in the peripheral nervous system. Biochemical studies of these diseases are still scarce and post‐mortem findings are often difficult to interpret on account of non‐specific pre‐mortem changes. Experimental models appear particularly useful, especially murine species which myelinate post‐natally in the brain. The discovery of mutations in the mouse whose phenotypic expression involves mainly myelination has provided a new tool to study the genetic pathology of myelin.