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Phenotypes Associated with This Genotype
Genotype
MGI:5558880
Allelic
Composition
Fbn1tm3.1Hcd/Fbn1+
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fbn1tm3.1Hcd mutation (1 available); any Fbn1 mutation (7 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• infiltration of activated B cells and plasma cells in the dermis
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• infiltration of activated B cells and plasma cells in the dermis
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• dermal polarization towards pro-inflammatory T helper cells
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• increased IL9, IL13 and IL22 in CD3+ dermal cells
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• in CD3+ dermal cells
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• in CD3+ dermal cells
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• increased anti-nuclear and anti-topoisomerase I antibody levels
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating

integument
• by 3 months
• however, skin condition is normalized by treatment with an integrin beta1 activating or TGF-beta-neutralizing antibody
• increased by 1 month
• however, skin condition is normalized by treatment with an integrin beta1 activating or TGF-beta-neutralizing antibody
• disorganized and excessive microfibrillar aggregates in the dermis with sparsely distributed elastin
• however, skin condition is normalized by treatment with an integrin beta1 activating or TGF-beta-neutralizing antibody
• decreased skin stretching (stiff skin)
• however, skin condition is normalized by treatment with an integrin beta1 activating or TGF-beta-neutralizing antibody

adipose tissue
• by 3 months
• however, skin condition is normalized by treatment with an integrin beta1 activating or TGF-beta-neutralizing antibody

hematopoietic system
• infiltration of activated B cells and plasma cells in the dermis
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• infiltration of activated B cells and plasma cells in the dermis
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating
• dermal polarization towards pro-inflammatory T helper cells
• however, auto-antibody levels are normalized by treatment with an integrin beta1 activating

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
systemic scleroderma DOID:418 OMIM:181750
J:206074


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)
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last database update
06/18/2019
MGI 6.14
The Jackson Laboratory