Myelin protein zero and membrane adhesion.
Protein zero (P0) is a member of the immunoglobulin gene superfamily (IgCAM) that is expressed at high levels in myelinated vertebrates in central (fish and amphibia) and peripheral (all species) myelin. This glycoprotein is the major adhesive component of peripheral myelin, where it mediates self-adhesion of the Schwann cell plasma membrane. Although the expression of P0 is naturally limited to Schwann cells, the molecular mechanisms of P0-mediated adhesion can be considered general and "obligatory" because, when expressed in a variety of cell lines, P0 induces strong intercellular adhesion. Modeling studies, X-ray crystallographic analysis, and experimental site-directed mutagenesis have provided excellent working models for understanding how P0 mediates adhesion at the atomic level. These models remain to be experimentally tested. However, in humans, certain mutations in P0 produce dysmyelinating disease, possibly due to disruptions in the predicted P0 lattice.
Spiryda
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LB
Department of Cell Biology, Mount Sinai School of Medicine, New York, New York 10029, USA. spiryda@msvax.mssm.edu
D000595:Amino Acid Sequence; D000818:Animals; D002448:Cell Adhesion / Q000502:physiology; D003711:Demyelinating Diseases / Q000378:metabolism; D019143:Evolution, Molecular; D005786:Gene Expression Regulation / Q000502:physiology*; D006801:Humans; D008969:Molecular Sequence Data; D018993:Myelin P0 Protein / Q000235:genetics*; D017386:Sequence Homology, Amino Acid
D018993:Myelin P0 Protein