Results 41 to 50 of about 10,140 (167)

Ca2+-dependent Interface Formation in Fibrillin-1 [PDF]

Journal of Biological Chemistry, 2005
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.
Jensen, SA, Corbett, A, Knott, V, Redfield, C, Handford, P   +4 more
openaire   +2 more sources

Association of FBN1 polymorphism with susceptibility of adolescent idiopathic scoliosis: a case-control study

BMC Musculoskeletal Disorders, 2022
Background Fibrillin-1 (FBN1) is an extracellular matrix glycoprotein essential to the structural component of microfibrils and FBN1 gene polymorphisms can be associated with adolescent idiopathic scoliosis (AIS) susceptibility.
Gustavo Borges Laurindo de Azevedo, Jamila Alessandra Perini, Antônio Eulálio Pedrosa Araújo Junior, Luis Antonio Medeiros Moliterno, Rodrigo Mantelatto Andrande, João Antonio Matheus Guimarães, Helton Luiz Aparecido Defino   +6 more
doaj   +1 more source

A Disulfide‐Sticker Strategy for Marine Adhesive Coatings: From Deciphering Self‐Assembly Mechanism to Functional Application in Hair Regeneration

Advanced Science, EarlyView.
A disulfide‐sticker strategy, coupled with Ca2+ coordination, drives the hierarchical self‐assembly of a recombinant scallop adhesive protein into fishnet‐like nanostructures, affording a coating with versatile wet adhesion and intrinsic antioxidant activity. This biocompatible coating markedly promotes hair regeneration by activating follicular niches,
Lulu Wang, Juan Yang, Xin Jiang, Zhanghui Zheng, Hongyu Wei, Na Li, Xiangqiang Chu, Weizhi Liu   +7 more
wiley   +1 more source

Protein Interaction Studies of MAGP-1 with Tropoelastin and Fibrillin-1 [PDF]

Journal of Biological Chemistry, 2001
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.
Jensen, Sacha A., Reinhardt, Dieter P., Gibson, Mark A., Weiss, Anthony S.   +3 more
openaire   +3 more sources

P3.11 INCREASED CAROTID PLAQUE OCCURRENCE IN MEN WITH THE FIBRILLIN-1 2–3 GENOTYPE

Artery Research, 2009
Background: Fibrillin-1 is an important constituent of the vascular wall and earlier studies have indicated an effect of the fibrillin-1 2–3 genotype on blood pressure as well as aortic stiffness.
R. DeBasso, B. Hedblad, J. Carlson, G. Berglund, T. Länne   +4 more
doaj   +1 more source

Compound heterozygous mutations in FBN1 in a large family with Marfan syndrome

Molecular Genetics & Genomic Medicine, 2020
Background Marfan syndrome (MFS) is a dominant monogenic disorder caused by mutations in fibrillin 1 (FBN1). Rarely, compound heterozygosity for FBN1 mutations has been described. Methods A large kindred with MFS was assessed clinically over decades, and
Aideen M. McInerney‐Leo, Jennifer West, Lawrie Wheeler, Paul J. Leo, Kim M. Summers, Lisa Anderson, Matthew A. Brown, Malcolm West, Emma L. Duncan   +8 more
doaj   +1 more source

Proteomic fingerprints of damage in extracellular matrix assemblies

Matrix Biology Plus, 2020
In contrast to the dynamic intracellular environment, structural extracellular matrix (ECM) proteins with half-lives measured in decades, are susceptible to accumulating damage.
Alexander Eckersley, Matiss Ozols, Ronan O'Cualain, Emma-Jayne Keevill, April Foster, Suzanne Pilkington, David Knight, Christopher E.M. Griffiths, Rachel E.B. Watson, Michael J. Sherratt   +9 more
doaj   +1 more source

Therapy for Myhre Syndrome: Goals, Misconceptions, and Current Agents

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre Syndrome (MYHRS, MIM #139210) is a rare, multisystem connective tissue disorder caused by recurrent heterozygous gain‐of‐function pathogenic variants in the SMAD4 gene, a key player in TGF‐β signaling and a regulator of extracellular matrix homeostasis.
Alessandro De Falco, Alfonso Manuel D'Alessio, Nicola Brunetti‐Pierri   +2 more
wiley   +1 more source

Review of the Molecular and Developmental Basis of Myhre Syndrome, Bench Research

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome (MS) is a connective‐tissue disorder within the acromelic dysplasia spectrum. It is characterized by congenital craniofacial, skeletal, cutaneous anomalies, respiratory, cardiovascular along with intellectual disability, deafness, and progressive fibrosis.
Camille Viaut, Valerie Cormier‐Daire
wiley   +1 more source

The distribution of fibrillin‐2 and LTBP‐2, and their co‐localisation with fibrillin‐1 in adult bovine tail disc [PDF]

Journal of Anatomy, 2011
AbstractWe investigated the distribution of fibrillin‐2 and LTBP‐2 (latent TGF‐β binding protein‐2) in the intervertebral disc of the adult bovine tail. The association of fibrillin‐2 and of LTBP‐2 with fibrillin‐1 was examined by dual immunofluorescence staining. Both fibrillin‐2 and LTBP‐2 were found extensively distributed in all regions of the disc
Li, B, Urban, J, Yu, J
openaire   +3 more sources