Interactions of the scid or beige mutations with the viable motheaten mutation.
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.
Dominique
V
V
Laboratoire d'immunologie, Université de Strasbourg 1, France.
Francis
L
L
D000818:Animals; D003433:Crosses, Genetic; D005260:Female; D005328:Fetal Viability / Q000235:genetics* / Q000502:physiology*; D006128:Growth / Q000502:physiology; D007136:Immunoglobulins / Q000097:blood; D008017:Life Expectancy / Q000639:trends; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D016513:Mice, SCID; D009026:Mortality / Q000639:trends; D012871:Skin Diseases / Q000235:genetics* / Q000401:mortality / Q000473:pathology*
D007136:Immunoglobulins