Accession Number:

ADA605019

Title:

Role of CTGF in White Matter Development in Tuberous Sclerosis

Descriptive Note:

Annual rept. 1 Feb 2013-31 Jan 2014

Corporate Author:

CHILDREN'S HOSPITAL CORP BOSTON MA

Personal Author(s):

Report Date:

2014-02-01

Pagination or Media Count:

14.0

Abstract:

Our preliminary results indicate that the connective tissue growth factor CTGF is necessary and sufficient to block the oligodendrocyte maturation. The Tsc1ffSynCre 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 Tsc1CTGFSynCre 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.

Subject Categories:

  • Anatomy and Physiology
  • Medicine and Medical Research

Distribution Statement:

APPROVED FOR PUBLIC RELEASE