Results 31 to 40 of about 1,608 (150)

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

Fibrillin-1 Misfolding and Disease

Antioxidants & Redox Signaling, 2006
Fibrillin-1 is a 350 kDa calcium-binding protein which assembles to form 10-12 nm microfibrils in the extracellular matrix (ECM). The structure of fibrillin-1 is dominated by two types of disulfide-rich motifs, the calcium- binding epidermal growth factor-like (cbEGF) and transforming growth factor beta binding protein-like (TB) domains.
Whiteman, P, Hutchinson, S, Handford, P
openaire   +3 more sources

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

Microenvironmental Regulation by Fibrillin-1

PLoS Genetics, 2012
Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes. A role for fibrillin-1 in specifying tissue microenvironments has not been elucidated, even though the concept that fibrillin-1 provides extracellular control of growth factor signaling is currently appreciated.
Sengle, Gerhard, Tsutsui, Ko, R. Keene, Douglas, Tufa, Sara F., Carlson, Eric J., Charbonneau, Noe L., Ono, Robert N., Sasaki, Takako, Wirtz, Mary K., Samples, John R., Fessler, Liselotte I., Fessler, John H., Sekiguchi, Kiyotoshi, Hayflick, Susan J., Sakai, Lynn Y.   +14 more
openaire   +5 more sources

Putative functions of extracellular matrix glycoproteins in secondary palate morphogenesis

Frontiers in Physiology, 2012
Cleft palate is a common birth defect in humans. Elevation and fusion of paired palatal shelves are coordinated by growth and transcription factors, and mutations in these can cause malformations.
Rocca ed'Amaro, Rolf eScheidegger, Susan eBlumer, Pawel ePazera, Christos eKatsaros, Daniel eGraf, Matthias eChiquet   +6 more
doaj   +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

A patient with Marfan syndrome in a general practitioner’s office

Pediatria i Medycyna Rodzinna, 2015
Marfan syndrome is a disorder of the connective tissue (fibrillins and elastins). It is an autosomal dominant disease associated with a defect of chromosome 15 which encodes fibrillin-1.
Magdalena Czerżyńska, Zuzanna Judyta Tyrakowska, Anna Wiśniewska, Emilia Rozwadowska   +3 more
doaj   +1 more source

Loss of POGLUT2/3‐mediated O‐glucosylation produces lung and aortic phenotypes reminiscent of fibrillin1 mutants

Developmental Dynamics, EarlyView.
Abstract Background Fibrillins provide a scaffold for elastic fiber formation, which enables lung recoil and aortic compliance. Abnormal fibrillin microfibrils, as in Marfan syndrome, lead to enlarged alveoli, vascular stiffening, and aneurysms. Our earlier studies suggested that fibrillin function depends on O‐glucosylation of its epidermal growth ...
Sanjiv Neupane, Angelina Almiroudis, Kaitlyn E. Donnelly, Robert S. Haltiwanger, Bernadette C. Holdener   +4 more
wiley   +1 more source

CD8+ T‐cells, CD86+ macrophages and TNF‐α signalling pathways are correlated with fetlock osteoarthritis in racehorses

Equine Veterinary Journal, EarlyView.
Abstract Background There is emerging evidence for the role of the immune system in osteoarthritis (OA) pathophysiology; however, little is known about how immune cells and the synovial transcriptome are altered in naturally occurring equine OA. Objectives To evaluate synovial fluid (SF) and synovial membrane (SM) immune cell populations and the SM ...
E. J. Secor, E. N. Chow, M. Thomas, L. Johnson, A. Siefert, B. Wagner, J. B. Engiles, H. L. Reesink   +7 more
wiley   +1 more source

The Supramolecular Organization of Fibrillin-Rich Microfibrils [PDF]

The Journal of Cell Biology, 2001
We propose a new model for the alignment of fibrillin molecules within fibrillin microfibrils. Automated electron tomography was used to generate three-dimensional microfibril reconstructions to 18.6-Å resolution, which revealed many new organizational details of untensioned microfibrils, including heart-shaped beads from which two arms emerge, and ...
Baldock, Clair, Koster, Abraham J., Ziese, Ulrike, Rock, Matthew J., Sherratt, Michael J.; id_orcid 0000-0003-4759-6617, Kadler, Karl E.; id_orcid 0000-0003-4977-4683, Adrian Shuttleworth, C., Kielty, Cay M.   +7 more
openaire   +4 more sources