Deficiency of SHP-1 protein-tyrosine phosphatase in "viable motheaten" mice results in retinal degeneration

Investigative Ophthalmology & Visual Science
Bonnie L LyonsLeonard D Shultz

Abstract

Viable motheaten mutant mice (abbreviated allele symbol me(v)) are deficient in Src-homology 2-domain phosphatase (SHP)-1, a critical negative regulator of signal transduction in hematopoietic cells. These mice exhibit immune dysfunction, hyperproliferation of myeloid cells, and regenerative anemia. This study focused on the role of SHP-1 in retinal homeostasis. Ophthalmoscopy, histology, transmission electron microscopy (TEM), electroretinography (ERG), immunohistochemistry, Western blot, bone marrow transplantation, and genetic crosses were performed for phenotypic characterization and functional studies of retinal degeneration (RD) in me(v)/me(v) mice. Fundus examinations of me(v)/me(v) mice revealed numerous, small white spots. Histologic examination demonstrated photoreceptor loss beginning at 3 weeks of age, and TEM revealed disorganization and reduction in the number of outer segments, as well as the presence of phagocytic cells in the subretinal space. Rod- and cone-mediated ERGs were abnormal. SHP-1 protein was expressed in mouse and human retinal lysates and was localized to the outer nuclear layer of the retina in me(v)/me(v) and control mice. Autoantibodies are not necessary for RD, as B-cell-deficient me(v)/me(v) I...Continue Reading

Citations

May 17, 2014·Glycobiology·Bettina Linnartz-GerlachHarald Neumann
May 27, 2014·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Ivo BuschmannKai Kappert
Aug 6, 2020·International Journal of Molecular Sciences·Huan LiaoHarald Neumann
Nov 19, 2020·Journal of Cellular and Molecular Medicine·Xiaonan ZhuangGezhi Xu
May 23, 2009·Clinical & Experimental Optometry : Journal of the Australian Optometrical Association·Jie Shen, Larry N Thibos

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