Mouse Genome Informatics
cn1
    Gt(ROSA)26Sortm1(Tgfbr1*)Crm/Gt(ROSA)26Sor+
Tg(Col1a2-cre/ERT)7Cpd/0

involves: 129S7/SvEvBrd * C57BL/6 * DBA/2
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
adipose tissue
• at 5 months after tamoxifen injections

cardiovascular system
• in tamoxifen-treated mice the walls of small arteries in the lungs, kidneys, and adrenal glands are thickened compared to oil-treated controls
• thickened vessels in the lungs show hypertrophy of the smooth muscle layers
• in tamoxifen-treated mice the walls of small arteries in the kidneys are thickened compared to oil-treated controls
• in tamoxifen-treated mice the walls of small arteries in the lungs are thickened compared to oil-treated controls
• thickened vessels in the lungs show hypertrophy of the smooth muscle layers
• seen in thickened small arteries in the lungs of tamoxifen-treated mice
• increased expression of Vwf in lung vessels of tamoxifen-treated mice suggestive of endothelial cell damage and similar to what is seen in human scleroderma patients

renal/urinary system
• in tamoxifen-treated mice the walls of small arteries in the kidneys are thickened compared to oil-treated controls
• increase in type I collagen deposition in the interstitium of tamoxifen-treated mice

growth/size
• in mice, injected with tamoxifen for 5 consecutive days starting at 2 weeks of age, at 5 months of age compared to control littermates

respiratory system
• in tamoxifen-treated mice the walls of small arteries in the lungs are thickened compared to oil-treated controls
• thickened vessels in the lungs show hypertrophy of the smooth muscle layers

muscle
• seen in thickened small arteries in the lungs of tamoxifen-treated mice

integument
• at 5 months after tamoxifen injections
• loss of hair on the back, head, chest, and sides and around the ears and nose in mice injected with tamoxifen for 5 consecutive days starting at 2 weeks of age
• tamoxifen-treated mice show progressive dermal fibrosis beginning by 1 month post injection
• a 2.3-fold in total collagen and disorganized collagen fibrils with increased variability in the size and shape of fibrils are seen in the skin of tamoxifen-treated mice
• dramatic increase in thickness at 5 months after tamoxifen injections
• at 5 months after tamoxifen injections
• increase in skin fragility at 5 months after tamoxifen injections
• skin feels thicker and rougher in tamoxifen treated mice compared to littermate controls

Mouse Models of Human Disease
OMIM IDRef(s)
Scleroderma, Familial Progressive 181750 J:134135