MBP/A0350

Myelin basic protein (MBP, also known as A0350) is the second most abundant protein in the myelin sheath of oligodendrocytes and Schwann cells in the central nervous system.

Myelin basic protein (MBP, also known as A0350) is the second most abundant protein in the myelin sheath of oligodendrocytes and Schwann cells in the central nervous system. MBP is responsible for adhesion of the cytosolic surfaces of multilayered compact myelin and may also have other functions such as intracellular signaling, interactions between the membrane and the cytoskeleton, and regulatory function on the expression of other myelin proteins. It can interact with actin, tubulin, calcium-calmodulin, and clathrin, and negatively charged lipids. Several studies have shown a role for antibodies against MBP in the pathogenesis of multiple sclerosis (MS). MS is a chronic inflammatory disease of the central nervous system characterized by demyelination and loss of neurological function. Knock-out mutations for this protein have been studied in the Shiverer mouse. This mouse shows an autosomal recessive mutation characterized by an almost total lack of central nervous system myelin. While small amounts of other myelin components are present in the brain of the Shiverer mouse, the four forms of MBP are not detectable. MBP mutants display moderate to severe brain hypomyelination due to an abnormal compaction of the myelin sheath. The mice also present tremor and convulsions of progressively increasing severity leading to early death. The homozygous Shiverer mice in whom the gene for MBP had been injected showed no more neurologic symptoms and survived normally.