About   Help   FAQ
Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
H2u
u variant
MGI:3834991
Summary 6 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
H2u/H2u
Tg(TCRA)B1Jg/0
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRA)B1Jg/J MGI:3835050
cx2
H2u/H2u
Tg(TCRA)B1Jg/0
Tg(TCRB)C14Jg/0
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRA)B1Jg Tg(TCRB)C14Jg MGI:3835052
cx3
H2u/H2u
Tg(TCRB)C14Jg/0
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRB)C14Jg/J MGI:3835051
cx4
H2u/H2u
Rag1tm1Mom/Rag1tm1Mom
Tg(Tcra19,Tcrb19)#Stl/0
either: (involves: 129S7/SvEvBrd * C57BL/6 * PL/J) or (involves: 129S7/SvEvBrd * C57BL/6 * C57BL/10 * PL/J) MGI:6305814
cx5
H2u/H2u
Tg(Tcra19,Tcrb19)#Stl/0
either: (involves: C57BL/6 * PL/J) or (involves: C57BL/6 * C57BL/10 * PL/J) MGI:6305810
cx6
H2u/H2u
Tg(TCRA)B1Jg/?
Tg(TCRB)C14Jg/?
involves: C57BL/6 * C57BL/10SnSg * DBA/2 * PL/J MGI:3835028


Genotype
MGI:3835050
cx1
Allelic
Composition
H2u/H2u
Tg(TCRA)B1Jg/0
Genetic
Background
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRA)B1Jg/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)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• the ratio of double negative to double-positive thymocytes is significantly higher (0.281) in these mice than in controls (0.016)
• thymus cellularity is reduced by 4 fold compared to controls
• double-negative thymocyte numbers are increased to 17.2% of all thymocytes from 1.2% in controls
• the absolute number of double negative thymocytes is increased despite reduced thymus cellularity
• double-positive thymocyte numbers are decreased to 61.8% of all thymocytes compared to 75.3% for controls
• the absolute number of single-positive CD4 thymocytes is decreased

immune system
• the ratio of double negative to double-positive thymocytes is significantly higher (0.281) in these mice than in controls (0.016)
• thymus cellularity is reduced by 4 fold compared to controls
• double-negative thymocyte numbers are increased to 17.2% of all thymocytes from 1.2% in controls
• the absolute number of double negative thymocytes is increased despite reduced thymus cellularity
• double-positive thymocyte numbers are decreased to 61.8% of all thymocytes compared to 75.3% for controls
• the absolute number of single-positive CD4 thymocytes is decreased

neoplasm
• 12.5 % of mice develop lymphoma characterized by CD8+CD4hi-low T cells
• the lymphoma is metastatic and transplantable

endocrine/exocrine glands
• the ratio of double negative to double-positive thymocytes is significantly higher (0.281) in these mice than in controls (0.016)
• thymus cellularity is reduced by 4 fold compared to controls
• 12.5 % of mice develop lymphoma characterized by CD8+CD4hi-low T cells
• the lymphoma is metastatic and transplantable




Genotype
MGI:3835052
cx2
Allelic
Composition
H2u/H2u
Tg(TCRA)B1Jg/0
Tg(TCRB)C14Jg/0
Genetic
Background
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRA)B1Jg Tg(TCRB)C14Jg
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 ratio of double negative to double-positive thymocytes is significantly higher (0.483) in these mice than in controls (0.016)
• the ratio of single-positive (both CD4+ and CD8+) thymocytes to double-positive thymocytes is significantly increased
• a greater proportion of the double-negative thymocytes present in E16 and E17 thymi are in the most mature (c-kit-CD44-CD25-) stage compared to controls
• double-negative thymocyte numbers are increased to 11.7% of all thymocytes from 1.2% in controls
• the absolute number of double-negative thymocytes is increased over wild-type and is twice that found in Tg(TCRA)B1Jg transgenic mice
• the appearance of double-positive T cells in the thymus first occurs on E16 compared to E17 for controls
• the appearance of double-positive T cells in the thymus first occurs on E16 compared to E17 for controls
• the percentage and absolute number of single-positive CD4 thymocytes is increased
• gamma-delta T cells are not detected in the thymus of E18 mice

neoplasm
• 21.9 % of mice develop lymphoma characterized by CD8+CD4hi-low T cells
• the lymphoma is metastatic and transplantable
• the lymphoma is not found in mice younger than 80 days of age and occurs with equal frequency to male and female mice

hematopoietic system
• the ratio of double negative to double-positive thymocytes is significantly higher (0.483) in these mice than in controls (0.016)
• the ratio of single-positive (both CD4+ and CD8+) thymocytes to double-positive thymocytes is significantly increased
• a greater proportion of the double-negative thymocytes present in E16 and E17 thymi are in the most mature (c-kit-CD44-CD25-) stage compared to controls
• double-negative thymocyte numbers are increased to 11.7% of all thymocytes from 1.2% in controls
• the absolute number of double-negative thymocytes is increased over wild-type and is twice that found in Tg(TCRA)B1Jg transgenic mice
• the appearance of double-positive T cells in the thymus first occurs on E16 compared to E17 for controls
• the appearance of double-positive T cells in the thymus first occurs on E16 compared to E17 for controls
• the percentage and absolute number of single-positive CD4 thymocytes is increased
• gamma-delta T cells are not detected in the thymus of E18 mice

endocrine/exocrine glands
• the ratio of double negative to double-positive thymocytes is significantly higher (0.483) in these mice than in controls (0.016)
• the ratio of single-positive (both CD4+ and CD8+) thymocytes to double-positive thymocytes is significantly increased
• 21.9 % of mice develop lymphoma characterized by CD8+CD4hi-low T cells
• the lymphoma is metastatic and transplantable
• the lymphoma is not found in mice younger than 80 days of age and occurs with equal frequency to male and female mice




Genotype
MGI:3835051
cx3
Allelic
Composition
H2u/H2u
Tg(TCRB)C14Jg/0
Genetic
Background
B10.PL-H2u H2-T18a/(73NS)Sn-Tg(TCRB)C14Jg/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2u mutation (6 available); any H2 mutation (274 available)
Tg(TCRB)C14Jg mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• a greater proportion of the double-negative thymocytes present in E16 and E17 thymi are in the most mature (c-kit-CD44-CD25-) stage compared to controls
• the appearance of double-positive T cells in the thymus first occurs on E15 compared to E17 for controls
• gamma-delta T cells are not detected in the thymus of E18 mice

hematopoietic system
• a greater proportion of the double-negative thymocytes present in E16 and E17 thymi are in the most mature (c-kit-CD44-CD25-) stage compared to controls
• the appearance of double-positive T cells in the thymus first occurs on E15 compared to E17 for controls
• gamma-delta T cells are not detected in the thymus of E18 mice




Genotype
MGI:6305814
cx4
Allelic
Composition
H2u/H2u
Rag1tm1Mom/Rag1tm1Mom
Tg(Tcra19,Tcrb19)#Stl/0
Genetic
Background
either: (involves: 129S7/SvEvBrd * C57BL/6 * PL/J) or (involves: 129S7/SvEvBrd * C57BL/6 * C57BL/10 * PL/J)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2u mutation (6 available); any H2 mutation (274 available)
Rag1tm1Mom mutation (49 available); any Rag1 mutation (92 available)
Tg(Tcra19,Tcrb19)#Stl mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• 100% of mice develop spontaneous autoimmune encephalomyelitis within 12 months
• onset and rate of progression of disease varies, with an average age of onset of 13 weeks
• cerebellum and spinal cord exhibit patchy lesions with mononuclear cell infiltrates
• activated myelin basic protein (MBP)-specific T cells are found in the brain but not in the peripheral lymphoid tissues

behavior/neurological
• weakness or paralysis of the hind legs is seen first and then extends to the front legs
• once hind legs are completely paralyzed, the disease progresses very rapidly

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
multiple sclerosis DOID:2377 OMIM:612594
OMIM:612595
OMIM:612596
J:19795




Genotype
MGI:6305810
cx5
Allelic
Composition
H2u/H2u
Tg(Tcra19,Tcrb19)#Stl/0
Genetic
Background
either: (involves: C57BL/6 * PL/J) or (involves: C57BL/6 * C57BL/10 * PL/J)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2u mutation (6 available); any H2 mutation (274 available)
Tg(Tcra19,Tcrb19)#Stl mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• about 11% and 14% of mice develop spontaneous autoimmune encephalomyelitis within 6 months and 12 months, respectively
• all mice develop a rapidly progressing form of experimental autoimmune encephalomyelitis after immunization with myelin basic protein (MBP)




Genotype
MGI:3835028
cx6
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)
Citing These Resources
Funding Information
Warranty Disclaimer & Copyright Notice
Send questions and comments to User Support.
last database update
09/27/2022
MGI 6.21
The Jackson Laboratory