Original paperFibrillin secretion and microfibril assembly by Marfan dermal fibroblasts
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Cited by (26)
Marfan Syndrome and Related Disorders
2013, Emery and Rimoin's Principles and Practice of Medical GeneticsClassical and neonatal Marfan syndrome mutations in fibrillin-1 cause differential protease susceptibilities and protein function
2011, Journal of Biological ChemistryCitation Excerpt :Loss of tissue integrity can result from deficiencies in the biogenesis of the microfibril/elastic fiber system and/or from excessive degradation of this system. Both mechanisms have been demonstrated with cells and tissues derived from patients with MFS (21–29). There is growing evidence that proteases play a significant role in the pathophysiology of MFS.
Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adaptor function to tropoelastin
2007, Journal of Biological ChemistryCitation Excerpt :For visualization of the fluorescent signals, an Axiocam microscope was used with AxioVision software version 3.1.2.1 (Zeiss). Extraction of Microfibrils and Identification of Associated Ligands—The experimental design for extraction and purification of microfibrils followed described procedures with some modifications (51, 52). Briefly, primary human dermal skin fibroblasts were grown for 6 weeks on a total culture area of ∼1000 cm2 in Dulbecco's modified Eagle's medium as described above.
Microfibrils at basement membrane zones interact with perlecan via fibrillin-1
2005, Journal of Biological ChemistryCitation Excerpt :In negative controls, the primary antibody was omitted and replaced by PBS. Basically, purification of microfibrils from cell cultures followed the procedure by Kielty et al. (70). Primary normal human skin fibroblasts (passage 2) were grown to postconfluence for 6 weeks on a total culture area of 600 cm2 under identical conditions as described under “Immunolabeling Experiments.”
Relaxin regulates fibrillin 2, but not fibrillin 1, mRNA and protein expression by human dermal fibroblasts and murine fetal skin
2003, Archives of Biochemistry and BiophysicsInteractions of Fibrillin-1 with Heparin/Heparan Sulfate, Implications for Microfibrillar Assembly
2001, Journal of Biological Chemistry