An essential role of MAG in mediating axon–myelin attachment in Charcot–Marie–Tooth 1A disease

摘要: Charcot-Marie-Tooth disease type 1A (CMT1A) is a hereditary demyelinating peripheral neuropathy caused by the duplication of PMP22 gene. Demyelination precedes occurrence clinical symptoms that correlate with axonal degeneration. It was postulated disturbed axon-glia interface contributes to altered myelination consequently leading In this study, we examined expression MAG and Necl4, two critical adhesion molecules are present at interface, in sural nerve biopsies CMT1A patients nerves mice overexpressing human PMP22, an animal model for CMT1A. We show increase strong decrease Necl4 as well mice. Expression analysis revealed strongly upregulated during maturation, whereas remains very low. Ablating results separation axons from their myelin sheath. Our data important contact CMT1A, suggest its increased has compensatory role pathology disease. Thus, demonstrate together other such sustaining integrity.