immune system
• very weak in vitro T cell proliferative responses to either whole AChR protein or peptides, indicating the supporting T cell repertoire never fully developed
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• purified splenocyte antigen presenting cells were impaired in their ability to stimulate highly primed wildtype CD4+ T cells in vitro with AChR protein, indicating impaired Ag presentation
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• reduced B cell responses to acetylcholine receptor
• despite repeated immunization of homozygous null mice with AChR protein, there was little B cell expansion
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• reduced anti-AChR IgG1, IgG2b, IgG2c subclass production in AChR-immunized homozygotes
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• reduced anti-AChR IgM production in AChR-immunized homozygotes
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• reduced humoral responses in AChR immunized homozygotes
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• production of IFN-gamma was suppressed in lymph node cells from AChR immunized homozygotes that were stimulated in vitro with AChR protein or peptides
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• production of IL-2 and IL-10 was suppressed in lymph node cells from AChR immunized homozygotes that were stimulated in vitro with AChR protein or peptides
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• homozygous null mice were markedly resistant to the development of experimental autoimmune myasthenia gravis (EAMG) (induced by acetylcholine receptor (AChR) immunization), with 17% of nulls developing EAMG compared to 75% of wildtype, and had significantly higher functional muscle acetylcholine receptors
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cardiovascular system
• during wound healing, microvessel development is impaired compared to in wild-type mice
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• endothelial cells exhibit impaired matrigel and collagen gel invasion compared with wild-type cells
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hematopoietic system
• reduced B cell responses to acetylcholine receptor
• despite repeated immunization of homozygous null mice with AChR protein, there was little B cell expansion
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• reduced anti-AChR IgG1, IgG2b, IgG2c subclass production in AChR-immunized homozygotes
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• reduced anti-AChR IgM production in AChR-immunized homozygotes
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• very weak in vitro T cell proliferative responses to either whole AChR protein or peptides, indicating the supporting T cell repertoire never fully developed
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