A disease-associated mutation in fibrillin-1 differentially regulates integrin-mediated cell adhesion. [PDF]
Fibrillins serve as scaffolds for the assembly of elastic fibers that contribute to the maintenance of tissue homeostasis and regulate growth factor signaling in the extracellular space.
Del Cid JS+9 more
europepmc +2 more sources
Since the discovery of bone morphogenetic proteins (BMPs) as pluripotent cytokines extractable from bone matrix, it has been speculated how targeting of BMPs to the extracellular matrix (ECM) modulates their bioavailability. Understanding these processes
Alexander P. Wohl+4 more
openalex +2 more sources
Ca2+-dependent Interface Formation in Fibrillin-1 [PDF]
The calcium-binding epidermal growth factor-like (cbEGF) domain is a common structural motif in extracellular and transmembrane proteins. K(d) values for Ca2+ vary from the millimolar to nanomolar range; however the molecular basis for this variation is poorly understood.
Sacha A. Jensen+4 more
openalex +4 more sources
Fibrillin-1 and −2 contain heparin-binding sites important for matrix deposition and that support cell attachment [PDF]
Claudio C Werneck
exaly +2 more sources
Acromicric dysplasia caused by a mutation of fibrillin 1 in a family: A case report. [PDF]
Shen R, Feng JH, Yang SP.
europepmc +2 more sources
Fibulin-5 interacts with fibrillin-1 molecules and microfibrils [PDF]
Fibulin-5 plays an important role in elastic fibre formation in vivo. We have investigated the molecular interactions between fibulin-5 and components of fibrillin-rich microfibrils which form a template for elastin. Fibulin-5 interacted in a dose-dependent manner with a fibrillin-1 N-terminal sequence and with tropoelastin, but not with MAGP-1 ...
Lyle J. Freeman+7 more
openalex +5 more sources
Macromolecular crowding enhances fibrillin-1 deposition in the extracellular matrix [PDF]
Biochemical and biophysical factors need consideration when modelling in vivo cellular behaviour using in vitro cell culture systems. One underappreciated factor is the high concentration of macromolecules present in vivo, which is typically not ...
B Satz-Jacobowitz+3 more
doaj +1 more source
Novel fibrillin-1 mutation with variable presentation as a thoracic aortic aneurysm and intramural hematoma. [PDF]
Brahmandam A, Vallabhajosyula P.
europepmc +3 more sources
Protein Interaction Studies of MAGP-1 with Tropoelastin and Fibrillin-1 [PDF]
Elastic fibers consist primarily of an amorphous elastin core associated with microfibrils, 10-12 nm in diameter, containing fibrillins and microfibril-associated glycoproteins (MAGPs). To investigate the interaction of MAGP-1 with tropoelastin and fibrillin-1, we expressed human MAGP-1 as a T7-tag fusion protein in Escherichia coli.
Sacha A. Jensen+3 more
openalex +6 more sources
Marfan syndrome variation of the POGLUT2 and POGLUT3 consensus sequence can produce aberrant fibrillin-1 O-glucosylation. [PDF]
Kegley NR+4 more
europepmc +2 more sources