Abnormal Schwann Cell-Axon Interaction In EBF-2 “Knock Out” Mice
Ebf-2 belongs to the Ebf gene family which has been implicated in various aspects of neural development. In the peripheral nervous system, Ebf-2 is highly expressed in immature Schwann cells. Ebf-2 expression is maintained in non myelin-forming Schwann cells, whereas it is down-regulated in myelin-forming Schwann cells. Although its exact role is still unclear, putative target genes include molecules involved in Schwann cell differentiation and maturation such as SCIP, N-CAM, L1, laminin, and the intermediate filament GFAP. Disruption of Ebf-2 affects nerve development and results in a defective Schwann cell-axon interaction. Deficient sorting of large calibre axons that lie in bundles in non-myelin-forming Schwann cells, associated in some cases with abnormal myelination, are the main characteristic features. This is likely the result of an aberrant communication between Schwann cells and axons.
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Document Type: Abstract
Affiliations: Dept. Neurosciences and DIBIT, San Raffele Scientific Institute.
Publication date: March 1, 2001