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Extracellular elastic fibres apply structure and mechanical elasticity to organs such as large arteries, lungs and skin 1. Elastic fibres are assembled through polymerization of tropoelastin monomers and loss of elastin is associated with aneurysmal degeneration of the aorta 2. The Fibulins are a ...
ORGANISM(S): Mus musculus 
Fibrillin-1, an extracellular matrix (ECM) protein encoded by FBN1 gene, serves as the microfibril scaffold crucial for elastic fiber formation and homeostasis in pliable tissue such as the skin. Besides causing Marfan syndrome, some mutations in FBN1 result in scleroderma, marked by hardened and th...
ORGANISM(S): Mus Musculus (mouse) 
Comparative gene array analyses of severe elastic fiber defects in late embryonic and newborn mouse aorta
Association of NOTCH3 with Elastic Fiber Dispersion in the Infrarenal Abdominal Aorta of Cynomolgus Monkeys
Elastic fibers provide reversible elasticity to the large arteries and are assembled during development when hemodynamic forces are increasing. Mutations in elastic fiber genes are associated with cardiovascular disease. Mice lacking expression of the elastic fiber genes elastin (Eln-/-), fibulin-4 ...
ORGANISM(S): Mus musculus 
2018-09-26 | GSE120465 | GEO
Gene expression profiling of newborn lung tissue revealed few changes in compound FGFR3/FGFR4 deficient mice, consistent with their normal lung morphology at birth, suggesting the sequence of events leading to the phenotype initiates after birth in this model. Profiling of 4 week-old lung tissues re...
ORGANISM(S): Mus musculus 
OBJECTIVE: The regional heterogeneity of vascular components and transcriptomes is an important determinant of aortic biology. This notion has been explored in multiple mouse studies. In the present study, we examined the regional heterogeneity of aortas in non-human primates. APPROACH AND RESULTS: ...
ORGANISM(S): Macaca fascicularis 
2023-03-22 | GSE227434 | GEO
Fibrillins play a critical role in tissue/organ development and cardiopulmonary function. Here we showed that O-glucosylation of the fibrillins’ epidermal growth factor-like repeats by protein O-glucosyltransferase 2 (POGLUT2) and POGLUT3 was indispensable for in vivo function of mouse fibrillins. L...
ORGANISM(S): Mus Musculus 
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