Interactions of the scid or beige mutations with the viable motheaten mutation.

Details

Serval ID
serval:BIB_E4C8A9938930
Type
Article: article from journal or magazin.
Collection
Publications
Title
Interactions of the scid or beige mutations with the viable motheaten mutation.
Journal
Autoimmunity
Author(s)
Dominique V., Francis L.
ISSN
0891-6934 (Print)
ISSN-L
0891-6934
Publication state
Published
Issued date
1995
Peer-reviewed
Oui
Volume
22
Number
4
Pages
199-207
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
The viable motheaten (mev) mice are characterized by a moth-eaten appearance of the coat, immunodeficiency, autoimmunity, generalized inflammatory disease, paws necroses, and early death. The target of the single point mev mutation is PTP1C, a protein tyrosine phosphatase whose deficient expression in hematopoietic cells should explain all phenotypic features of mev mice, particularly their autoimmune and inflammatory pathologies. In order to evaluate their role in the development of the mev mouse disease, we constructed mevscid congenics to probe the impact of autoimmunity and mevbeige congenics to probe the impact of elastase and cathepsine G neutrophil activities. Both mevscid and mevbeige mice were nearly equivalent to mev mice with regards to moth-eaten appearance, paw necroses and early death. Thus, autoimmunity does neither initiate nor substantially enhance the mev mouse syndrome. Moreover, the beige mutation-linked deficiency of protease activity of neutrophils is unable to significantly reduce the mev mutation-dependent inflammatory pathology.

Keywords
Animals, Crosses, Genetic, Female, Fetal Viability/genetics, Fetal Viability/physiology, Growth/physiology, Immunoglobulins/blood, Life Expectancy/trends, Male, Mice, Mice, Inbred C57BL, Mice, Mutant Strains, Mice, SCID, Mortality/trends, Skin Diseases/genetics, Skin Diseases/mortality, Skin Diseases/pathology
Pubmed
Web of science
Create date
06/10/2016 10:33
Last modification date
20/08/2019 16:08
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