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Phenotypes Associated with This Genotype
Genotype
MGI:3835028
Allelic
Composition
H2u/H2u
Tg(TCRA)B1Jg/?
Tg(TCRB)C14Jg/?
Genetic
Background
involves: C57BL/6 * C57BL/10SnSg * DBA/2 * PL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2u mutation (6 available); any H2 mutation (274 available)
Tg(TCRA)B1Jg mutation (1 available)
Tg(TCRB)C14Jg mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• the double-positive population in the thymus is reduced by almost half
• majority of mice have CD4 T cells expressing the transgenic TCR
• however, in some mice T cells expressing the transgenic TCR do not mature
• in other mice, transgenic T cell mature but fail to express the CD4 coreceptor
• there is about a 3-fold increase in the percentage of CD4 single-positive thymoyctes compared to wild-type controls
• CD4 T cells numbers are also significantly increased in the spleen
• decreased CD8-positive T cells are found in the spleen
• there is also a decrease in CD8 single-positive thymocytes
• CD4 T cells proliferate strongly and secrete cytokines in response to peptide from the first 20 amino acids of myelin basic protein (MBP) displayed in the context of H2u
• mice are much more susceptible to experimental autoimmune encephalomyelitis induced by MBP immunization
• 29 of 32 mice develop EAE after induction with MBP-adjuvant compared to 10 of 28 for controls
• severity of EAE in transgenic mice is greater than controls
• EAE could also be induced by administration of pertussis toxin or LPS in these mice but not in controls
• a small percentage of mice develop spontaneous EAE
• immunization with MBP peptide leads to about a 100-fold higher levels of anti-MBP antibodies compared to immunized wild-type mice

behavior/neurological
• spontaneous experimental autoimmune encephalomyelitis (EAE) can occur in these mice when housed under non-sterile conditions
• 12 mice within a 10 month period developed hind limb paralysis
• this paralysis was associated with infiltrates into the spinal cord white matter
• between 14 and 44% of mice develop a limp tail during a 4 week window starting at six weeks of age
• some of these mice progress to a hind limb paralysis
• transgenic mice kept in a germ-free facility did not develop this spontaneous EAE

hematopoietic system
• the double-positive population in the thymus is reduced by almost half
• majority of mice have CD4 T cells expressing the transgenic TCR
• however, in some mice T cells expressing the transgenic TCR do not mature
• in other mice, transgenic T cell mature but fail to express the CD4 coreceptor
• there is about a 3-fold increase in the percentage of CD4 single-positive thymoyctes compared to wild-type controls
• CD4 T cells numbers are also significantly increased in the spleen
• decreased CD8-positive T cells are found in the spleen
• there is also a decrease in CD8 single-positive thymocytes
• CD4 T cells proliferate strongly and secrete cytokines in response to peptide from the first 20 amino acids of myelin basic protein (MBP) displayed in the context of H2u

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
multiple sclerosis DOID:2377 OMIM:612594
OMIM:612595
OMIM:612596
J:92991


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