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| Nomenclature |
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Symbol:
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Faslpr-cg
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Name:
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Fas (TNF receptor superfamily member 6);
lymphoproliferation complementing gld
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MGI ID: |
MGI:1856335 |
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Synonyms: |
lprcg |
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Gene:
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Fas
Location:
Chr19:34290659-34327770 bp, + strand
Genetic Position: Chr19,
29.48 cM
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Mutation
origin |
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Strain of Origin:
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CBA/KlJms
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Mutation
description |
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Allele
Type: | |
Spontaneous |
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Mutation: | |
Single point mutation |
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Mutation details: A T-to-A transversion point mutation at nucleotide 786 results in replacement by asparagine of a highly conserved isoleucine in the cytoplasmic region of the encoded protein. (J:1181)
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Inheritance: | |
Recessive |
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Phenotypes
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View phenotypes for all genotypes (concatenated display).
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Disease models
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| Find Mice (IMSR) |
Mouse strains and cell lines available from the
International Mouse Strain Resource
(IMSR)
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Notes |
This mutation, which produces massive lymphadenopathy in homozygotes, occurred spontaneously in a subline of the inbred strain CBA/KlJms maintained at the Institute of Medical Science in Tokyo. Faslpr-cg complemented both Faslpr and the generalized lymphoproliferative disease Faslgld mutation in that double heterozygotes with either mutation had lymphadenopathy. However, further crosses showed the new mutation to be an allele at Faslpr (J:24805). Like Faslpr and Faslgld mutant homozygotes, CBA-Faslpr-cg/Faslpr-cg mutants produce antibodies to some nuclear antigens, such as dsDNA, ssDNA, and poly(ADP-ribose); however, they do not produce anti-erythrocyte antibodies. Although they exhibit lymphoid cell infiltration around blood vessels in lung, liver, and kidney, they lack the immune-complex glomerulonephritis, vasculitis, and interstitial pneumonia characteristic of Faslpr homozygotes (J:616). Faslpr-cg homozygotes on the MRL genetic background developed glomerulonephritic lesions similar to those of MRL/MpJ-Faslpr mutants, although at a lower frequency, suggesting MRL/MpJ background genes control this aspect of the disease (J:1753). Studies of Faslpr and Faslpr-cg homozygotes in athymic nude (Hfh11nu/Hfh11nu) mice show that the thymus is critical for lymphadenopathy and autoimmunity (J:582).
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| References |
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Original: |
J:24805
Matsuzawa A et al.,
"A new allele of the lpr locus, lprcg, that complements the gld gene in induction of lymphadenopathy in the mouse."
J Exp Med 1990 Feb 1;171(2):519-31
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All: |
24 reference(s)
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Analysis Tools
