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

被引:26
|
作者
Bihl, F
Larivière, L
Qureshi, ST
Flaherty, L
Malo, D [1 ]
机构
[1] Montreal Gen Hosp, Ctr Study Host Resistance, Montreal, PQ H3G 1A4, Canada
[2] McGill Univ, Dept Human Genet, Montreal, PQ H3G 1Y6, Canada
[3] McGill Univ, Dept Med, Montreal, PQ H3G 1Y6, Canada
[4] Yale Univ, Sch Med, New Haven, CT 06520 USA
[5] NY State Dept Hlth, Wadsworth Ctr, Albany, NY 12208 USA
基金
加拿大健康研究院; 英国医学研究理事会;
关键词
rodent; endotoxin shock; infectious immunity; bacteria; lipopolysaccharide; molecular biology;
D O I
10.1038/sj.gene.6363732
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
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. Genes and Immunity (2001) 2, 56-59.
引用
收藏
页码:56 / 59
页数:4
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