Analysis Tools
immune system
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• Th1 cells are enriched in the CNS more than 2-fold
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• Th17 cells are enriched in the CNS 3-fold
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• ataxic mice show a higher expression of cytokines in the brain than paralytic relapsed mice
• paralytic relapsed mice show a higher expression of cytokines in the spinal cord than ataxic mice
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• mice develop spontaneous experimental autoimmune encephalomyelitis (EAE) at high frequency
• in the eight backcross generation to SJL/J, more than 80% of females, and more than 60% of males, develop EAE within 160 days
• typically, EAE starts with relapsing-remitting (RR) in females, with the first attacks often resolving completely, followed by further bouts
• in a set of mice, the initial bout is dominated by ataxia, with affected mice unable to walk along a straight line, deviating and falling to the side, but not showing limb weakness or paralysis
• in many cases, mice recover completely until a first relapse, commonly showing hind limb paralysis
• later disease episodes mainly show classical paralytic EAE
• RR disease is more common in females than males
• minority of females develop progressive EAE from the beginning whereas, more than 50% of males develop primary progressive EAE
• EAE lesions show prominent deposits of Ig and some activated complement
• mice treated with anti-CD20 antibody to deplete B cells, show suppression of RR-EAE when started at young age
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• mice produce anti-MOG autoantibodies of endogenous Ighb allotype; anti-MOG Igs are mainly IgG1 and IgG2a/b isotypes, with very little IgM
• autoantibodies are seen from 5 weeks of life and persist up to 9-10 weeks
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• inflamed CNS tissue contains CD4+ and CD8+ lymphocytes and substantial numbers of B cells expressing CD19 or B220
• inflammatory regions are characterized by numerous CD3+ T cells in the middle of Mac3+ activated macrophage-like cells and are embedded in large areas of demyelination and axon destruction
• most CD4+ T cells infiltrating the CNS are activated
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• ataxic mice exhibit large inflammatory and demyelinated lesions in cerebellum and brain stem
• mice showing conventional EAE exhibit lesions distributed throughout the spinal cord, brain stem, and optic nerve
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nervous system
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• inflamed CNS tissue contains CD4+ and CD8+ lymphocytes and substantial numbers of B cells expressing CD19 or B220
• inflammatory regions are characterized by numerous CD3+ T cells in the middle of Mac3+ activated macrophage-like cells and are embedded in large areas of demyelination and axon destruction
• most CD4+ T cells infiltrating the CNS are activated
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• ataxic mice exhibit large inflammatory and demyelinated lesions in cerebellum and brain stem
• mice showing conventional EAE exhibit lesions distributed throughout the spinal cord, brain stem, and optic nerve
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• ataxic mice exhibit large inflammatory and demyelinated lesions in cerebellum and brain stem
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behavior/neurological
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• in a set of mice, the initial bout of autoimmune encephalomyelitis is seen as ataxia
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• seen in relapsing mice and in later disease episodes
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hematopoietic system
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• Th1 cells are enriched in the CNS more than 2-fold
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• Th17 cells are enriched in the CNS 3-fold
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homeostasis/metabolism
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• ataxic mice show a higher expression of cytokines in the brain than paralytic relapsed mice
• paralytic relapsed mice show a higher expression of cytokines in the spinal cord than ataxic mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| multiple sclerosis | DOID:2377 |
OMIM:612594 OMIM:612595 OMIM:612596 |
J:149511 | |
