An adhesion test system based on Schneider cells to determine genotype- phenotype correlations for mutated P0 proteins

Arif Bülent Ekici, Christina Fuchs, Eva Nelis, Rainer Hillenbrand, Melitta Schachner, Christine Van Broeckhoven, Bernd Rautenstrauss

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

Myelin protein zero (MPZ, P0) is well known as the adhesion molecule responsible for the comparison of the myelin sheath of peripheral nerves. Mutations are linked to Charcot-Marie-Tooth syndrome type 1B (CMT1B) and the more severe Dejerine-Sottas syndrome (DSS). Three mutations leading to phenotypes of increasing severity of (Ser34del/CMT1B, Ser34Cys/DSS, INS663GC/DSS) were expressed in S2 insect cells and resulted in a decreased adhesion capability in correlation with their respective phenotypes.

Original languageEnglish (US)
Pages (from-to)117-119
Number of pages3
JournalGenetic Analysis - Biomolecular Engineering
Volume14
Issue number4
DOIs
StatePublished - Oct 1998

All Science Journal Classification (ASJC) codes

  • Applied Microbiology and Biotechnology
  • Genetics

Keywords

  • Adhesion
  • Heterologous expression
  • Homophilic binding
  • Immunoglobulin superfamily
  • P0
  • Schneider cells

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