Mouse Genome Informatics
hm
    Wastm1Sbs/Wastm1Sbs
129S6/SvEvTac-Wastm1Sbs/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
homeostasis/metabolism
• mutants older than 6 months of age exhibit proteinuria (J:180407)

immune system
• elevation in serum IgA, both in young and old mutants (J:180407)
• B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5 (J:180407)
• reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants (J:180407)
• mutants exhibit higher titers of circulating IgA-containing complexes (J:180407)
• mutants exhibit increased glomerular IgA deposition in the kidneys (J:180407)
• mutants exhibit increased glomerular IgM and C3 complement deposition in the kidneys (J:180407)
• mutants develop proliferative glomerulonephritis similar to human IgA nephropathy (J:180407)

renal/urinary system
• mutants older than 6 months of age exhibit proteinuria (J:180407)
• mutants exhibit renal injury with increasing severity with advancing age (J:180407)
• mutants develop proliferative glomerulonephritis similar to human IgA nephropathy (J:180407)
• mutants older than 6 months of age exhibit hump-like mesangial and paramesangial deposits (J:180407)
• mutants older than 6 months of age, exhibit increased mesangial cellularity with or without endocapillary proliferation (J:180407)
• mutants older than 6 months of age, exhibit increased matrix deposition with or without endocapillary proliferation (J:180407)

hematopoietic system
• elevation in serum IgA, both in young and old mutants (J:180407)
• B cells exhibit increased IgA production after stimulation with LPS, TGF-beta1, IL-4, and IL-5 (J:180407)
• reduced ratio of sialylated and galactosylated IgA in the serum, indicating that glycosylation of IgA is abnormal in mutants (J:180407)
• mutants exhibit higher titers of circulating IgA-containing complexes (J:180407)
• mutants exhibit increased glomerular IgA deposition in the kidneys (J:180407)
• mutants exhibit increased glomerular IgM and C3 complement deposition in the kidneys (J:180407)

Mouse Models of Human Disease
OMIM IDRef(s)
Iga Nephropathy, Susceptibility to, 1; IGAN1 161950 J:180407
Wiskott-Aldrich Syndrome; WAS 301000 J:180407