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

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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.

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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
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  2. L Larivière
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  3. ST Qureshi
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  4. L Flaherty
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  5. D Malo
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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.

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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

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

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