Skip NavigationSkip to Content
Return Return to Search Results

Disruption of axo-glial junctions causes cytoskeletal disorganization and degeneration of Purkinje neuron axons

  1. Author:
    Garcia-Fresco, G. P.
    Sousa, A. D.
    Pillai, A. M.
    Moy, S. S.
    Crawley, J. N.
    Tessarollo, L.
    Dupree, J. L.
    Bhat, M. A.

  2. Author Address

    Univ N Carolina, Sch Med, Curriculum Neurobiol, Chapel Hill, NC 27599 USA. Univ N Carolina, Sch Med, Dept Cell & Mol Physiol, Chapel Hill, NC 27599 USA. Univ N Carolina, Sch Med, Dept Psychiat, Chapel Hill, NC 27599 USA. Univ N Carolina, Sch Med, Ctr Neurosci, Chapel Hill, NC 27599 USA. Univ N Carolina, Sch Med, Neurodev Disorders Res Ctr, Chapel Hill, NC 27599 USA. NIMH, Intramural Res Program, Bethesda, MD 20892 USA. NCI, Neural Dev Grp, Frederick, MD 21702 USA. Virginia Commonwealth Univ, Dept Anat & Neurobiol, Richmond, VA 23298 USA.;Bhat, MA, Univ N Carolina, Sch Med, Curriculum Neurobiol, Chapel Hill, NC 27599 USA.;manzoor_bhat@med.unc.edu
    1. Year: 2006
    2. Date: Mar
  2. Journal: Proceedings of the National Academy of Sciences of the United States of America
    1. 103
    2. 13
    3. Pages: 5137-5142
  4. Type of Article: Article
  5. ISSN: 0027-8424
  1. Abstract:

    Axo-glial junctions (AGJs) play a critical role in the organization and maintenance of molecular domains in myelinated axons. Neurexin IV/Caspr1/paranodin (NCP1) is an important player in the formation of AGJs because it recruits a paranodal complex implicated in the tethering of glial proteins to the axonal membrane and cytoskeleton. Mice deficient in either the axonal protein NCP1 or the glial ceramide galactosyltransferase (CGT) display disruptions in AGJs and severe ataxia. in this article, we correlate these two phenotypes and show that both NCP1 and CGT mutants develop large swellings accompanied by cytoskeletal disorganization and degeneration in the axons of cerebellar Purkinje neurons. We also show that all spectrin is part of the paranodal complex and that, although not properly targeted, this complex is still formed in CGT mutants. Together, these findings establish a physiologically relevant link between AGJs and axonal cytoskeleton and raise the possibility that some neurodegenerative disorders arise from disruption of the AGJs.

    See More

  1. Keywords:

External Sources

  1. View Full Text
  2. DOI: 10.1073/pnas.0601082103
  3. View record in Web of Science®
  4. WOS: 000236472500062

Library Notes

  1. No notes added.
NCI at Frederick

You are leaving a government website.

This external link provides additional information that is consistent with the intended purpose of this site. The government cannot attest to the accuracy of a non-federal site.

Linking to a non-federal site does not constitute an endorsement by this institution or any of its employees of the sponsors or the information and products presented on the site. You will be subject to the destination site's privacy policy when you follow the link.

ContinueCancel