Mimecan/osteoglycin-deficient mice have collagen fibril abnormalities.

摘要: PURPOSE To study the role of mimecan, a member small leucine-rich proteoglycans (SLRPs) gene family and one major components cornea other connective tissues, mice that lack functional mimecan were generated characterized. METHODS Mimecan-deficient by gene-targeting using standard techniques. Mice genotyped Southern blot analysis. The absence transcripts was confirmed Northern Corneal clarity examined slit lamp biomicroscopy. strength skin evaluated biomechanical fragility test. Collagen morphology in preparations from mimecan-null control wild-type analyzed transmission electron microscopy. diameter collagen fibrils these tissues determined morphometric RESULTS lacking appear to develop normally, are viable fertile. In controlled laboratory environment they do not display an evident pathological phenotype compared wild type mice. Examination corneal measurements thickness show no significant changes cornea. However, test revealed moderate reduction tensile mutant Ultrastructural analyses show, on average, thicker both average 31.84+/-0.322 nm, versus 22.40+/-0.296 nm their litter-mates. most pronounced increase fibril found mice, who demonstrated 130.33+/-1.769 78.82+/-1.157 addition, size variability altered detected dorsal tail CONCLUSIONS results present demonstrate similar members SLRP family, has regulating fibrillogenesis vivo. Further studies, such as challenges, evaluation potential compensation proteins (including family), generation double-knockouts will be necessary fully uncover physiological functions