Phenotypes for B4galt1 MGI:6306320 MGI Mouse

renal/urinary system

albuminuria ( J:117891 )

hematuria ( J:117891 )

• hematuria is seen in some mice and increases with age

abnormal renal glomerulus morphology ( J:117891 )

• 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

glomerulonephritis ( J:117891 )

• glomeruli show predominantly IgA, and some IgG, IgM, and C3 mesangial deposits, with deposits specifically detected in the paramesangial areas

abnormal glomerular mesangium morphology ( J:117891 )

• 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

expanded mesangial matrix ( J:117891 )

glomerulosclerosis ( J:117891 )

• glomerular sclerosis is seen at 8 weeks and progresses with age

immune system

increased IgA level ( J:117891 )

• 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

abnormal IgM level ( J:117891 )

• 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