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Biochemical and biophysical research communications v.494 no.3/4, 2017년, pp.504 - 510   SCI SCIE
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The L1 adhesion molecule normalizes neuritogenesis in Rett syndrome-derived neural precursor cells

Yoo, Myungsik (W. M. Keck Center for Collaborative Neuroscience and Department of Cell Biology and Neuroscience, Rutgers University, 604 Allison Road, Piscataway, NJ 08845, USA ); Carromeu, Cassiano (School of Medicine, Department of Pediatrics/Rady Children's Hospital San Diego, Department of Cellular and Molecular Medicine, Stem Cell Program, 9500 Gilman Drive, La Jolla, CA 92093, MC 0695, USA ); Kwon, Ohyoon (W. M. Keck Center for Collaborative Neuroscience and Department of Cell Biology and Neuroscience, Rutgers University, 604 Allison Road, Piscataway, NJ 08845, USA ); Muotri, Alysson (School of Medicine, Department of Pediatrics/Rady Children's Hospital San Diego, Department of Cellular and Molecular Medicine, Stem Cell Program, 9500 Gilman Drive, La Jolla, CA 92093, MC 0695, USA ); Schachner, Melitta (W. M. Keck Center for Collaborative Neuroscience and Department of Cell Biology and Neuroscience, Rutgers University, 604 Allison Road, Piscataway, NJ 08845, USA );
  • 초록  

    Abstract Therapeutic intervention is an important need in ameliorating the severe consequences of Rett Syndrome (RTT), a neurological disorder caused by mutations in the X-linked gene methyl-CpG-binding protein-2 (MeCP2). Following previously observed morphological defects in induced pluripotent stem cell (iPSC)-derived neurons obtained from female RTT patients, we hypothesized that transfection with the L1 cell adhesion molecule (L1) could contribute to normalizing a pathological male cell system bearing a nonsense mutation of MeCP2. We found a decreased expression of L1 in RTT iPSCs-derived neural precursor cells (RTT NPCs) and decreased neuritogenesis. Expression of wild-type MeCP2 in RTTNPCs revealed a positive correlation between the levels of MeCP2 and L1, and normalization of cell survival. Expression of L1 in RTTNPCs enhanced neuritogenesis and soma size. Knock-down of MeCP2 in wild type NPCs reduced neuritogenesis. L1 expression is regulated by the MeCP2 promoter. These results suggest that a deficiency in L1 may partially account for RTT phenotypes. Highlights L1 expression is downregulated in RTT NPCs. MeCP2 expression correlates positively with L1 expression levels. MeCP2 and L1 expression in RTT NPCs normalizes their impaired neuritogenesis. MeCP2 expression rescues the reduced substrate adhesion of RTT NPCs. MeCP2 binds to an E-box domain in the human L1 promoter, enhancing transcription.


  • 주제어

    MeCP2 .   L1CAM .   RTT syndrome .   iPSCs .   Neuritogenesis.  

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