title: Severe defects in immunity and hematopoiesis caused by SHP-1 protein-tyrosine-phosphatase deficiency.
abstract: Spontaneous mouse mutations that cause severe immunodeficiency or autoimmunity are invaluable tools with which to investigate the mammalian immune system. Mutations at the 'motheaten' locus result in severe immunological dysfunction due to disruption of the structural gene encoding Src-homology 2-domain phosphatase-1 (SHP-1). This natural model for a specific protein-tyrosine-phosphatase deficiency is being widely utilized to determine the role of SHP-1 in the negative regulation of multiple signaling pathways in a number of hematopoietic lineages.
authors.affiliation: Jackson Laboratory, Bar Harbor, ME 04609, USA. lds@aretha.jax.org
authors.forename: L D
authors.initials: LD
authors.lastname: Shultz
authors.forename: T V
authors.initials: TV
authors.lastname: Rajan
authors.forename: D L
authors.initials: DL
authors.lastname: Greiner
mesh_terms: D000818:Animals; D015971:Gene Expression Regulation, Enzymologic / Q000276:immunology; D006410:Hematopoiesis / Q000276:immunology*; D007153:Immunologic Deficiency Syndromes / Q000201:enzymology* / Q000235:genetics / Q000276:immunology; D047908:Intracellular Signaling Peptides and Proteins; D009154:Mutation / Q000276:immunology; D054645:Protein Phosphatase 1; D054592:Protein Tyrosine Phosphatase, Non-Receptor Type 11; D053824:Protein Tyrosine Phosphatase, Non-Receptor Type 6; D017027:Protein Tyrosine Phosphatases / Q000172:deficiency* / Q000276:immunology / Q000378:metabolism
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; D054645:Protein Phosphatase 1; D054592:Protein Tyrosine Phosphatase, Non-Receptor Type 11; D053824:Protein Tyrosine Phosphatase, Non-Receptor Type 6; D017027:Protein Tyrosine Phosphatases; C516725:Ptpn11 protein, mouse; C509248:Ptpn6 protein, mouse