Results 61 to 70 of about 2,560 (174)

Fibrillins Can Co-assemble in Fibrils, but Fibrillin Fibril Composition Displays Cell-specific Differences [PDF]

Journal of Biological Chemistry, 2003
Fibrillins are microfibril-forming extracellular matrix macromolecules that modulate skeletal development. In humans, mutations in fibrillins result in long bone overgrowth as well as other distinct phenotypes. Whether fibrillins form independent microfibrillar networks or can co-polymerize, forming a single microfibril, is not known.
Charbonneau, Noe L., Dzamba, Bette J., Ono, Robert N., Keene, Douglas R., Corson, Glen M., Reinhardt, Dieter P., Sakai, Lynn Y.   +6 more
openaire   +2 more sources

Molecular insights in the pathogenesis of classical Ehlers-Danlos syndrome from transcriptome-wide expression profiling of patients' skin fibroblasts.

PLoS ONE, 2019
Classical Ehlers-Danlos syndrome (cEDS) is a dominant inherited connective tissue disorder mainly caused by mutations in the COL5A1 and COL5A2 genes encoding type V collagen (COLLV), which is a fibrillar COLL widely distributed in a variety of connective
Nicola Chiarelli, Giulia Carini, Nicoletta Zoppi, Marco Ritelli, Marina Colombi   +4 more
doaj   +1 more source

The hidden science of haptics: A pedagogical review of tactile evaluation in cosmetics

International Journal of Cosmetic Science, EarlyView.
This review explores the physiological basis of tactile perception and critically examines classical and emerging sensory methods used in cosmetics, advocating for broader methodological integration and recognition of sensory analysis in the field. Abstract Sensory analysis is a cornerstone of cosmetic development, yet remains underrepresented in peer ...
Morgane Postec, Daria Terescenco, Ecaterina Gore   +2 more
wiley   +1 more source

Fibrillin-1 and fibrillin-2 in human embryonic and early fetal development

Matrix Biology, 2002
The extracellular glycoproteins fibrillin-1 and fibrillin-2 are major components of connective tissue microfibrils. Mutations in the fibrillin-1 and fibrillin-2 genes are responsible for the phenotypical manifestations of Marfan syndrome and congenital contractural arachnodactyly respectively, which emphasizes their essential roles in developmental ...
Quondamatteo, Fabio, Reinhardt, D. P., Charbonneau, N. L., Pophal, G., Sakai, L. Y., Herken, R.   +5 more
openaire   +3 more sources

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., Kadler, Karl E., Adrian Shuttleworth, C., Kielty, Cay M.   +7 more
openaire   +4 more sources

Matrix Metalloproteinases and Glaucoma Treatment. [PDF]

, 2020
Matrix metalloproteinases (MMPs) are a family of proteolytic enzymes that degrade extracellular matrix (ECM) components such as collagen and have important roles in multiple biological processes, including development and tissue remodeling, both in ...
Dibas, Mohammed, Robinson, Michael R, Stamer, W Daniel, Weinreb, Robert N   +3 more
core  

Lifespan Regulation by Evolutionarily Conserved Genes Essential for Viability [PDF]

, 2011
Evolutionarily conserved mechanisms that control aging are predicted to have prereproductive functions in order to be subject to natural selection. Genes that are essential for growth and development are highly conserved in evolution, but their role in ...
Curran, Sean Patrick, Ruvkun, Gary B.
core   +1 more source

The tympanic covering layer contributes to basilar membrane elasticity potentially influencing human frequency resolution and speech perception

Journal of Anatomy, EarlyView.
In this study we show for the first time that the human basilar membrane contains elastin produced by the so‐called tympanic covering layer. It is believed to play an important functional role in human cochlear tuning, particularly low frequencies linked to our remarkable speech and music perception.
Wei Liu, Claudia Steinacher, Alexander Glueckert, Rudolf Glueckert, Hao Li, Karin Staxäng, Anneliese Schrott‐Fischer, Hanif M. Ladak, Sumit Agrawal, Helge Rask‐Andersen   +9 more
wiley   +1 more source

Fibrillin-3 expression in human development [PDF]

Matrix Biology, 2011
Fibrillin proteins are the major components of extracellular microfibrils found in many connective tissues. Fibrillin-1 and fibrillin-2 are well studied and mutations in these proteins cause a number of fibrillinopathies including Marfan syndrome and congenital contractural arachnodactyly, respectively.
Sabatier, Laetitia, Miosge, Nicolai, Hubmacher, Dirk, Lin, Guoqing, Davis, Elaine C., Reinhardt, Dieter P.   +5 more
openaire   +3 more sources

Chronic tendon pathology: molecular basis and therapeutic implications [PDF]

, 2005
Tendons are frequently affected by chronic pain or rupture. Many causative factors have been implicated in the pathology, which until relatively recently was under-researched and poorly understood.
Riley, GP
core   +1 more source