Role of CTGF in White Matter Development in Tuberous Sclerosis

Abstract

Our preliminary results indicate that the connective tissue growth factor (CTGF) is necessary and sufficient to block the oligodendrocyte maturation. The Tsc1f/f;SynCre+ mice show reduced myelination compared to wild type controls. We showed that by visualizing the oligodendrocytes by PLP promoter driven GFP expression. In addition to this finding, we now demonstrate the decrease in mature oligodendrocyte number in vivo by staining another mature oligodendrocyte marker, CC1. To determine the role of CTGF in vivo, we are in process of generating Tsc1;CTGF;SynCre+ mice. In order to investigate the effect of CTGF on oligodendrocyte maturation, we proposed to treat the oligodendrocytes with different domains of CTGF (Module I to IV). We are in process of generating HA-tagged versions of full length, and separate domains of CTGF. So far we cloned the full length, Module I and Module I and II of CTGF. In addition to the downstream effects of CTGF on oligodendrocytes, we are investigating the molecular mechanisms of upregulation of CTGF in TSC-deficient neurons. We focus on two main pathways, which were previously shown to regulate expression of CTGF.

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Document Details

Document Type
Technical Report
Publication Date
Feb 01, 2014
Accession Number
ADA605019

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  • Mustafa Şahin

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DTIC Thesaurus Topics

  • Autism
  • Biological Staining And Labeling
  • Biomedical Research
  • Brain
  • Breast Cancer
  • Connective Tissue
  • Culture Media
  • Diseases And Disorders
  • Gene Expression
  • Growth Factors
  • Health Services
  • Maturation
  • Neoplasms
  • Peptide Growth Factors
  • Sclerosis
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