Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Brief Communication
  • Published:

LPS-hyporesponsiveness of mnd mice is associated with a mutation in Toll-like receptor 4

Genes & Immunity volume 2, pages 56–59 (2001)Cite this article

Abstract

Toll-like receptors (Tlrs) are transmembrane proteins that have recently been shown to play a critical role in the innate immune recognition of microbial constituents. Among this family, Tlr4 is a crucial signal transducer for lipopolysaccharide (LPS), the major component of the Gram-negative bacteria outer cell membrane. In this paper, we report that C57BL/6.KB2-mnd mice, a model of neuronal ceroid lipofuscinosis, do not respond to LPS. This defect is associated with a spontaneous mutation in Tlr4 consisting of a large insertion within exon 2 predicting a frameshift mutation and a truncated protein.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Similar content being viewed by others

Author information

Authors and Affiliations

  1. Department of Human Genetics, McGill University, Montréal, Québec, H3G 1Y6, Canada

    F Bihl & D Malo

  2. Centre for the Study of Host Resistance, Montreal General Hospital, Montréal, Québec, H3G 1A4, Canada

    F Bihl, L Larivière & D Malo

  3. Yale University School of Medicine, New Haven, 06520, CT, USA

    ST Qureshi

  4. NY State Department of Health, Wadsworth Center, Albany, NY 12208, New York, USA

    L Flaherty

  5. Department of Medicine, McGill University, Montréal, Québec, H3G 1Y6, Canada

    D Malo

Authors
  1. F Bihl
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. L Larivière
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. ST Qureshi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. L Flaherty
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. D Malo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to D Malo.

Additional information

DM is a Scholar of Fonds de la Recherche en Santé du Québec (FRSQ) and a Scientist from the Canadian Institutes for Health Research. STQ is a recipient of a Clinician Scientist Award from MRC and a Career Award in the Biomedical Sciences from the Burroughs Wellcome Fund. This work was supported by research grant from the Medical Research Council of Canada.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bihl, F., Larivière, L., Qureshi, S. et al. LPS-hyporesponsiveness of mnd mice is associated with a mutation in Toll-like receptor 4. Genes Immun 2, 56–59 (2001). https://doi.org/10.1038/sj.gene.6363732

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.gene.6363732

Keywords

This article is cited by

Search

Advanced search

Quick links