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Phenotypes Associated with This Genotype
Genotype
MGI:5300824
Allelic
Composition
Tg(Tnfsf13b)1Fma/Tg(Tnfsf13b)1Fma
Genetic
Background
involves: C57BL/6 * DBA/2
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No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

immune system
• mutants exhibit an increased percentage of B cells in mucosal lymphoid tissues, including the gut lamina propria and the mesenteric lymph nodes, and in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit B cell hyperplasia in the spleen (J:178227)
• mutants exhibit an increased percentage of B cells in mucosal lymphoid tissues, including the gut lamina propria and the mesenteric lymph nodes, and in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit B cell hyperplasia in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of transitional 2 B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of transitional 2 B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of splenic marginal zone B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of splenic marginal zone B cells (J:178227)
• serum IgA level in germ-free mutants is decreased about 100-fold compared to specific-pathogen free mutants but is similar to those for germ-free wild-type mutants (J:178227)
• germ-free mutants do not exhibit increased IgA in plasma cells in the gut and deposition of IgA in the glomeruli as is seen in conventionally housed specific pathogen free mutants (J:178227)
• serum IgA level in germ-free mutants is decreased about 100-fold compared to specific-pathogen free mutants but is similar to those for germ-free wild-type mutants (J:178227)
• germ-free mutants do not exhibit increased IgA in plasma cells in the gut and deposition of IgA in the glomeruli as is seen in conventionally housed specific pathogen free mutants (J:178227)
• while IgM and IgA levels are elevated in mutants, serum IgG levels and antinuclear IgG antibodies are not elevated in mutants (J:178227)
• while IgM and IgA levels are elevated in mutants, serum IgG levels and antinuclear IgG antibodies are not elevated in mutants (J:178227)
• by 8 months of age, most mutants exhibit IgA mesangial deposition in the glomeruli, however little C4 deposition is seen (J:178227)
• IgA serum levels are elevated by 8 weeks of age in males and remain at this elevated level until 6 to 8 months of age, at which point, IgA levels begin to drop (J:178227)
• in most females, serum IgA levels do not rise (J:178227)
• serum IgA in mutants is aberrantly glycosylated, with reduced amounts of terminal galactose and sialic acid, is polymeric and is not associated with the secretory component (J:178227)
• germ-free mutants colonized with a limited commensal microbiota called altered Schaedler flora (ASF) at 6 weeks of age exhibit an increase in serum IgA levels that continue to increase until about 3.5 weeks and then stabilize compared to wild-type mice colonized with ASF (J:178227)
• conventionally housed specific pathogen free mutants colonized with ASF exhibit widespread dissemination of IgA+ plasma cells in the periphery while germ-free mutants housed in a barrier facility do not show IgA+ plasma cells in the periphery after colonization, indicating that the hyper-IgA syndrome is influenced by ongoing-commensual-dependent signaling in the intestine (J:178227)
• by 8 months of age, most mutants exhibit IgA mesangial deposition in the glomeruli, however little C4 deposition is seen (J:178227)
• IgA serum levels are elevated by 8 weeks of age in males and remain at this elevated level until 6 to 8 months of age, at which point, IgA levels begin to drop (J:178227)
• in most females, serum IgA levels do not rise (J:178227)
• serum IgA in mutants is aberrantly glycosylated, with reduced amounts of terminal galactose and sialic acid, is polymeric and is not associated with the secretory component (J:178227)
• germ-free mutants colonized with a limited commensal microbiota called altered Schaedler flora (ASF) at 6 weeks of age exhibit an increase in serum IgA levels that continue to increase until about 3.5 weeks and then stabilize compared to wild-type mice colonized with ASF (J:178227)
• conventionally housed specific pathogen free mutants colonized with ASF exhibit widespread dissemination of IgA+ plasma cells in the periphery while germ-free mutants housed in a barrier facility do not show IgA+ plasma cells in the periphery after colonization, indicating that the hyper-IgA syndrome is influenced by ongoing-commensual-dependent signaling in the intestine (J:178227)
• mutants with mild proteinuria at 6-8 months of age, exhibit mesangial deposits of IgM in the glomeruli (J:178227)
• IgM serum levels are elevated by 8 weeks of age (J:178227)
• mutants with mild proteinuria at 6-8 months of age, exhibit mesangial deposits of IgM in the glomeruli (J:178227)
• IgM serum levels are elevated by 8 weeks of age (J:178227)
• mutants develop severe and fatal nephritis by 17 months of age (J:178227)
• males develop nephritis faster than females and this gender bias is thought to result from sexual dimorphism in expression in the kidneys of the alpha1-antitrypsin driven transgene (J:178227)
• mutants develop severe and fatal nephritis by 17 months of age (J:178227)
• males develop nephritis faster than females and this gender bias is thought to result from sexual dimorphism in expression in the kidneys of the alpha1-antitrypsin driven transgene (J:178227)

homeostasis/metabolism
• by 6 months of age, mutants exhibit modest albuminuria that gets worse with age (J:178227)
• by 6 months of age, mutants exhibit modest albuminuria that gets worse with age (J:178227)

hematopoietic system
• mutants exhibit an increased percentage of B cells in mucosal lymphoid tissues, including the gut lamina propria and the mesenteric lymph nodes, and in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit B cell hyperplasia in the spleen (J:178227)
• mutants exhibit an increased percentage of B cells in mucosal lymphoid tissues, including the gut lamina propria and the mesenteric lymph nodes, and in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit B cell hyperplasia in the spleen (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of transitional 2 B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of transitional 2 B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of splenic marginal zone B cells (J:178227)
• both specific pathogen free and germ-free mutants exhibit expanded percentages of splenic marginal zone B cells (J:178227)
• serum IgA level in germ-free mutants is decreased about 100-fold compared to specific-pathogen free mutants but is similar to those for germ-free wild-type mutants (J:178227)
• germ-free mutants do not exhibit increased IgA in plasma cells in the gut and deposition of IgA in the glomeruli as is seen in conventionally housed specific pathogen free mutants (J:178227)
• serum IgA level in germ-free mutants is decreased about 100-fold compared to specific-pathogen free mutants but is similar to those for germ-free wild-type mutants (J:178227)
• germ-free mutants do not exhibit increased IgA in plasma cells in the gut and deposition of IgA in the glomeruli as is seen in conventionally housed specific pathogen free mutants (J:178227)
• while IgM and IgA levels are elevated in mutants, serum IgG levels and antinuclear IgG antibodies are not elevated in mutants (J:178227)
• while IgM and IgA levels are elevated in mutants, serum IgG levels and antinuclear IgG antibodies are not elevated in mutants (J:178227)
• by 8 months of age, most mutants exhibit IgA mesangial deposition in the glomeruli, however little C4 deposition is seen (J:178227)
• IgA serum levels are elevated by 8 weeks of age in males and remain at this elevated level until 6 to 8 months of age, at which point, IgA levels begin to drop (J:178227)
• in most females, serum IgA levels do not rise (J:178227)
• serum IgA in mutants is aberrantly glycosylated, with reduced amounts of terminal galactose and sialic acid, is polymeric and is not associated with the secretory component (J:178227)
• germ-free mutants colonized with a limited commensal microbiota called altered Schaedler flora (ASF) at 6 weeks of age exhibit an increase in serum IgA levels that continue to increase until about 3.5 weeks and then stabilize compared to wild-type mice colonized with ASF (J:178227)
• conventionally housed specific pathogen free mutants colonized with ASF exhibit widespread dissemination of IgA+ plasma cells in the periphery while germ-free mutants housed in a barrier facility do not show IgA+ plasma cells in the periphery after colonization, indicating that the hyper-IgA syndrome is influenced by ongoing-commensual-dependent signaling in the intestine (J:178227)
• by 8 months of age, most mutants exhibit IgA mesangial deposition in the glomeruli, however little C4 deposition is seen (J:178227)
• IgA serum levels are elevated by 8 weeks of age in males and remain at this elevated level until 6 to 8 months of age, at which point, IgA levels begin to drop (J:178227)
• in most females, serum IgA levels do not rise (J:178227)
• serum IgA in mutants is aberrantly glycosylated, with reduced amounts of terminal galactose and sialic acid, is polymeric and is not associated with the secretory component (J:178227)
• germ-free mutants colonized with a limited commensal microbiota called altered Schaedler flora (ASF) at 6 weeks of age exhibit an increase in serum IgA levels that continue to increase until about 3.5 weeks and then stabilize compared to wild-type mice colonized with ASF (J:178227)
• conventionally housed specific pathogen free mutants colonized with ASF exhibit widespread dissemination of IgA+ plasma cells in the periphery while germ-free mutants housed in a barrier facility do not show IgA+ plasma cells in the periphery after colonization, indicating that the hyper-IgA syndrome is influenced by ongoing-commensual-dependent signaling in the intestine (J:178227)
• mutants with mild proteinuria at 6-8 months of age, exhibit mesangial deposits of IgM in the glomeruli (J:178227)
• IgM serum levels are elevated by 8 weeks of age (J:178227)
• mutants with mild proteinuria at 6-8 months of age, exhibit mesangial deposits of IgM in the glomeruli (J:178227)
• IgM serum levels are elevated by 8 weeks of age (J:178227)

renal/urinary system
• by 6 months of age, mutants exhibit modest albuminuria that gets worse with age (J:178227)
• by 6 months of age, mutants exhibit modest albuminuria that gets worse with age (J:178227)
• kidneys show expanded glomeruli and glomerular capillary thickening but no sclerosis (J:178227)
• with age, mutants display severe, global, and diffuse deposition of hyaline material in the glomeruli (J:178227)
• kidneys show expanded glomeruli and glomerular capillary thickening but no sclerosis (J:178227)
• with age, mutants display severe, global, and diffuse deposition of hyaline material in the glomeruli (J:178227)
• kidneys show rare mild mesangial cell proliferation but no glomerular sclerosis, tubular atrophy, or interstitial perivascular infiltration (J:178227)
• kidneys show rare mild mesangial cell proliferation but no glomerular sclerosis, tubular atrophy, or interstitial perivascular infiltration (J:178227)
• kidneys exhibit segmental multifocal expansion of the mesangial matrix with hyaline materials (J:178227)
• kidneys exhibit segmental multifocal expansion of the mesangial matrix with hyaline materials (J:178227)
• mild focal tubular atrophy (J:178227)
• mild focal tubular atrophy (J:178227)
• mutants exhibit impaired kidney function by 14-15 months of age that is preceded by an elevation in IgA at 8-10 months of age (J:178227)
• mutants exhibit impaired kidney function by 14-15 months of age that is preceded by an elevation in IgA at 8-10 months of age (J:178227)
• mutants develop severe and fatal nephritis by 17 months of age (J:178227)
• males develop nephritis faster than females and this gender bias is thought to result from sexual dimorphism in expression in the kidneys of the alpha1-antitrypsin driven transgene (J:178227)
• mutants develop severe and fatal nephritis by 17 months of age (J:178227)
• males develop nephritis faster than females and this gender bias is thought to result from sexual dimorphism in expression in the kidneys of the alpha1-antitrypsin driven transgene (J:178227)

Mouse Models of Human Disease
OMIM ID Ref(s)
Iga Nephropathy, Susceptibility to, 1; IGAN1 161950 J:178227


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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory