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Phenotypes Associated with This Genotype
Genotype
MGI:5644517
Allelic
Composition
Tg(H2-Kb-Tcra,-Tcrb)1640Kurs/0
Genetic
Background
SJL.FVB-Tg(H2-Kb-Tcra,-Tcrb)1640Kurs
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype

Severe infiltration, demyelination, and axonal damage in Tg(H2-Kb-Tcra,-Tcrb)1640Kurs/0 cerebellum and spinal cord

immune system
• Th1 cells are enriched in the CNS more than 2-fold
• Th17 cells are enriched in the CNS 3-fold
• 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
• 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
• 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
• 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
• 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

nervous system
• 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
• 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
• ataxic mice exhibit large inflammatory and demyelinated lesions in cerebellum and brain stem

behavior/neurological
• in a set of mice, the initial bout of autoimmune encephalomyelitis is seen as ataxia
• seen in relapsing mice and in later disease episodes

hematopoietic system
• Th1 cells are enriched in the CNS more than 2-fold
• Th17 cells are enriched in the CNS 3-fold

homeostasis/metabolism
• 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

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
multiple sclerosis DOID:2377 OMIM:612594
OMIM:612595
OMIM:612596
J:149511


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
08/03/2022
MGI 6.21
The Jackson Laboratory