Skip NavigationSkip to Content
Return Return to Search Results

Als-Linked Sod1 Mutant G85r Mediates Damage to Astrocytes and Promotes Rapidly Progressive Disease With Sod1-Containing Inclusions

  1. Author:
    Bruijn, L. I.
    Becher, M. W.
    Lee, M. K.
    Anderson, K. L.
    Jenkins, N. A.
    Copeland, N. G.
    Sisodia, S. S.
    Rothstein, J. D.
    Borchelt, D. R.
    Price, D. L.
    Clevel, D. W.

  2. Author Address

    Bruijn LI UNIV CALIF SAN DIEGO LUDWIG INST CANC RES LA JOLLA, CA 92093 USA UNIV CALIF SAN DIEGO DEPT MED LA JOLLA, CA 92093 USA UNIV CALIF SAN DIEGO DEPT NEUROSCI LA JOLLA, CA 92093 USA JOHNS HOPKINS UNIV SCH MED DEPT PATHOL BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED DEPT NEUROL BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED DEPT NEUROSCI BALTIMORE, MD 21205 USA JOHNS HOPKINS UNIV SCH MED DEPT NEUROPATHOL BALTIMORE, MD 21205 USA NCI MAMMALIAN GENET LAB ADV BIOSCI LABS RES PROGRAM FREDERICK, MD 21701 USA
    1. Year: 1997
  2. Journal: Neuron
    1. 18
    2. 2
    3. Pages: 327-338
  4. Type of Article: Article
  1. Abstract:

    High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and G37R were previously shown to mediate disease in mice through an acquired toxic property. We report here that even low levels of another mutant, G85R, cause motor neuron disease characterized by an extremely rapid clinical progression, without changes in SOD1 activity. Initial indicators of disease are astrocytic inclusions that stain intensely with SOD1 antibodies and ubiquitin and SOD1-containing aggregates in motor neurons, features common with some cases of SOD1 mutant-mediated ALS. Astrocytic inclusions escalate markedly as disease progresses, concomitant with a decrease in the glial glutamate transporter (GLT-1). Thus, the G85R SOD1 mutant mediates direct damage to astrocytes, which may promote the nearly synchronous degeneration of motor neurons. [References: 39]

    See More

  1. Keywords:

External Sources

  1. No sources found.

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