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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
B4galt1tm1Yiw
targeted mutation 1, Yoichiro Iwakura
MGI:2450889
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
B4galt1tm1Yiw/B4galt1tm1Yiw involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:6306320
hm2
B4galt1tm1Yiw/B4galt1tm1Yiw involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:3620087
ht3
B4galt1tm1Yiw/B4galt1+ involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:6306325
ht4
B4galt1tm1Yiw/B4galt1+ involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:3620088


Genotype
MGI:6306320
hm1
Allelic
Composition
B4galt1tm1Yiw/B4galt1tm1Yiw
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
B4galt1tm1Yiw mutation (3 available); any B4galt1 mutation (41 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• hematuria is seen in some mice and increases with age
• glomerulomegaly
• acellular eosinophilic lesions are seen near the vascular pole of the glomerulus
• glomerular lesions are classified into minor glomerular abnormality, segmental lesion and global lesion
• about 75% of glomeruli are affected at 8-13 weeks of age, with increased global lesions with age
• glomeruli show predominantly IgA, and some IgG, IgM, and C3 mesangial deposits, with deposits specifically detected in the paramesangial areas
• Mason's trichrome staining suggests deposition of immune complexes in the mesangial area
• mice exhibit predominantly IgA, and some IgG, IgM, and C3 mesangial deposits
• glomerular sclerosis is seen at 8 weeks and progresses with age

immune system
• glomeruli show predominantly IgA mesangial deposits, with deposits specifically detected in the paramesangial areas
• serum IgA levels increase with age with increased polymeric forms and completely absent beta4-galactosylation and sialylation of the N-glycans on the serum IgA
• serum IgM levels are about 2-fold higher than in heterozygous mice at 12-13 weeks of age but are about half of that in heterozygotes at 24-28 weeks of age
• glomeruli show some IgM mesangial deposits
• glomeruli show predominantly IgA, and some IgG, IgM, and C3 mesangial deposits, with deposits specifically detected in the paramesangial areas

homeostasis/metabolism
• hematuria is seen in some mice and increases with age

hematopoietic system
• glomeruli show predominantly IgA mesangial deposits, with deposits specifically detected in the paramesangial areas
• serum IgA levels increase with age with increased polymeric forms and completely absent beta4-galactosylation and sialylation of the N-glycans on the serum IgA
• serum IgM levels are about 2-fold higher than in heterozygous mice at 12-13 weeks of age but are about half of that in heterozygotes at 24-28 weeks of age
• glomeruli show some IgM mesangial deposits

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
IgA glomerulonephritis DOID:2986 OMIM:161950
J:117891




Genotype
MGI:3620087
hm2
Allelic
Composition
B4galt1tm1Yiw/B4galt1tm1Yiw
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
B4galt1tm1Yiw mutation (3 available); any B4galt1 mutation (41 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• only 20% survive beyond 16 weeks of age
• about half die before 4 weeks of age

immune system
• total white blood cell counts in the peripheral blood are increased 2.2-fold
• however, the number of colony forming units formed by bone marrow cells in the presence of G-CSF or IL3 are similar to wild-type
• neutrophil counts are increased 2.3-fold
• lymphocyte counts are increased 2.5-fold
• expression of selectin ligand and binding of P-selectin to splenic monocytes is severely reduced; however lymphocyte homing to peripheral lymph nodes is normal
• 3 and 6 days after injury macrophage infiltration is decreased
• expression of selectin ligand and binding of P-selectin to splenic neutrophils is severely reduced
• 6 and 24 hours after injury neutrophil infiltration is reduced and binding of infiltrated neutrophils to P-selectin is weak compared to binding of heterozygous controls
• zymosan-induced ear swelling is significantly reduced and neutrophil trafficking into the ear is not induced
• contact hypersensitivity induced by 2,4,6-trinitrochlorobenzene in the ear lobe is partially suppressed and neutrophil trafficking is also reduced
• delayed type hypersensitivity induced by methylated bovine serum albumin in the foot pad is partially suppressed and leukocyte infiltration into the dermis of the footpad is blocked

cardiovascular system
• 6 days after injury vascular density at the wound site is decreased

digestive/alimentary system
• increased proliferation of epithelial cells
• differentiation of villi is abnormal with premature expression of maltase and sucrase-isomaltase, increased maltase and isomaltase activities, decreased lactase activity, and delayed or absent sucrase activity
• in the small intestine the crypts are enlarged 3- to 4-fold and the mitotic index is increased about 4-fold

growth/size/body
• at 4 weeks of age average body weight is 60-70% that of wild-type mice

reproductive system
• litter sizes from crosses of homozygous females to heterozygous males or heterozygous females to homozygous males are reduced compared to wild-type crosses

hematopoietic system
• erythroid precursors, granulocytes and megakaryocytes are seen in the spleen and liver
• number of colony forming units formed by bone marrow cells in the presence of EPO is increased
• slight but significant
• slight but significant
• slight but significant
• total white blood cell counts in the peripheral blood are increased 2.2-fold
• however, the number of colony forming units formed by bone marrow cells in the presence of G-CSF or IL3 are similar to wild-type
• neutrophil counts are increased 2.3-fold
• lymphocyte counts are increased 2.5-fold
• expression of selectin ligand and binding of P-selectin to splenic monocytes is severely reduced; however lymphocyte homing to peripheral lymph nodes is normal
• 3 and 6 days after injury macrophage infiltration is decreased
• expression of selectin ligand and binding of P-selectin to splenic neutrophils is severely reduced
• 6 and 24 hours after injury neutrophil infiltration is reduced and binding of infiltrated neutrophils to P-selectin is weak compared to binding of heterozygous controls

endocrine/exocrine glands
• in the small intestine the crypts are enlarged 3- to 4-fold and the mitotic index is increased about 4-fold
• lactose is absent from the milk and females can not rear their offspring

cellular
• mitotic index is increased 4-fold in intestinal crypt epithelium
• 3 and 6 days after injury macrophage infiltration is decreased

homeostasis/metabolism
• 3 and 6 days after injury (full thickness excisional wounds on the dorsal skin) wound closure is delayed, the rate of re-epithelialization is reduced, macrophage infiltration is decreased, and collagen synthesis is significantly lower; however by 14 days wounds have healed to the same extent as in heterozygous controls
• 3, but not 6, days after injury chemokine expression at the wound site is reduced
• 6 and 24 hours after injury neutrophil infiltration is reduced

integument
• lactose is absent from the milk and females can not rear their offspring
• thickening of the horny layer is also seen in the lesions
• granular layer hyperplasia is seen in the skin lesions
• prickle cell hyperplasia is seen in the skin lesions and keratohyalin granules are abnormally deposited in the prickle cell layer
• most develop severe skin lesions within a few days of birth, these lesions shed horny cells for about 2 weeks but eventually heal




Genotype
MGI:6306325
ht3
Allelic
Composition
B4galt1tm1Yiw/B4galt1+
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
B4galt1tm1Yiw mutation (3 available); any B4galt1 mutation (41 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• mice show a slight increase in segmental lesions in glomeruli
• about 75% of glomeruli exhibit minor glomerular abnormality at older (47-48 weeks) ages




Genotype
MGI:3620088
ht4
Allelic
Composition
B4galt1tm1Yiw/B4galt1+
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
B4galt1tm1Yiw mutation (3 available); any B4galt1 mutation (41 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• litter sizes from crosses of homozygous females to heterozygous males or heterozygous females to homozygous males are reduced compared to wild-type crosses

endocrine/exocrine glands
• lactose concentration is about 50% of wild-type

integument
• lactose concentration is about 50% of wild-type





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last database update
03/12/2024
MGI 6.23
The Jackson Laboratory