Results 81 to 90 of about 19,374 (248)

Fibrillin–integrin interactions in health and disease

open access: yesBiochemical Society Transactions, 2008
Human fibrillin-1 is the major structural protein of extracellular matrix 10–12 nm microfibrils. It has a disulfide-rich modular organization which consists primarily of cbEGF (Ca2+-binding epidermal growth factor-like) domains and TB (transforming growth factor β-binding protein-like) domains.
Jovanovic, J   +4 more
openaire   +5 more sources

Characteristics of the tomato chromoplast revealed by proteomic analysis [PDF]

open access: yes, 2010
Chromoplasts are non-photosynthetic specialized plastids that are important in ripening tomato fruit (Solanum lycopersicum) since, among other functions, they are the site of accumulation of coloured compounds.
Barsan, Cristina   +9 more
core   +3 more sources

Dill extract induces elastic fiber neosynthesis and functional improvement in the ascending aorta of aged mice with reversal of age-dependent cardiac hypertrophy and involvement of lysyl oxidase-like-1 [PDF]

open access: yes, 2020
Elastic fibers (90% elastin, 10% fibrillin-rich microfibrils) are synthesized only in early life and adolescence mainly by the vascular smooth muscle cells through the cross-linking of its soluble precursor, tropoelastin.
Boyle, Walter A   +7 more
core   +3 more sources

Higher blood pressure in elderly hypertensive females, with increased arterial stiffness and blood pressure in females with the Fibrillin-1 2/3 genotype

open access: yesBMC Cardiovascular Disorders, 2020
Background Elderly patients have a relatively high cardiovascular risk due to increased arterial stiffness, elevated blood pressure and decreased amounts of elastin in the arteries.
Ida Åström Malm   +4 more
doaj   +1 more source

Characterisation of spinal ligaments in the embryonic chick

open access: yesJournal of Anatomy, EarlyView.
Use of the embryonic chick provides an opportunity to examine the maturation of spinal ligaments during development, to inform anatomical defects in the spine. Abstract Ligaments are important connective tissues within the musculoskeletal system that connect bone to bone and provide support and stability.
Sarah Hennigan   +2 more
wiley   +1 more source

The role of calcium in the organization of fibrillin microfibrils

open access: yesFEBS Letters, 1993
The microfibrillar glycoprotein fibrillin has a multidomain structure which contains forty‐three epidermal growth factor‐like motifs with calciumbinding consensus sequences. We have utilized intact microfibrils isolated from human dermal fibroblast cultures to investigate the putative influence of bound calcium on microfibrillar organization and ...
Kielty, CM, Shuttleworth, CA
openaire   +4 more sources

Adamtsl2 deletion results in bronchial fibrillin microfibril accumulation and bronchial epithelial dysplasia: A novel mouse model providing insights on geleophysic dysplasia [PDF]

open access: yes, 2015
Mutations in the secreted glycoprotein ADAMTSL2 cause recessive geleophysic dysplasia (GD) in humans and Musladin–Lueke syndrome (MLS) in dogs. GD is a severe, often lethal, condition presenting with short stature, brachydactyly, stiff skin, joint ...
Apte, Suneel S.   +4 more
core   +2 more sources

Pathogenic Mechanisms of Bicuspid Aortic Valve Aortopathy

open access: yesFrontiers in Physiology, 2017
Bicuspid aortic valve (BAV) is the most common congenital valvular defect and is associated with ascending aortic dilation (AAD) in a quarter of patients. AAD has been ascribed both to the hemodynamic consequences of normally functioning and abnormal BAV
Noor M. Yassine   +2 more
doaj   +1 more source

Cooperative Mechanism of ADAMTS/ ADAMTSL and Fibrillin-1 in the Marfan Syndrome and Acromelic Dysplasias

open access: yesFrontiers in Genetics, 2021
The term “fibrillinopathies” gathers various diseases with a wide spectrum of clinical features and severity but all share mutations in the fibrillin genes.
Pauline Arnaud   +5 more
doaj   +1 more source

Genotype–Phenotype Correlations, Treatment, and Prognosis of Children With Early‐Onset (Neonatal) Marfan Syndrome

open access: yesClinical Genetics, Volume 108, Issue 2, Page 134-145, August 2025.
Early‐onset Marfan syndrome (eoMFS) is a rare disorder with atrioventricular valve insufficiency being the most severe symptom. We propose to regard eoMFS as a spectrum, ranging from a severe disorder life‐threatening already before or immediately after birth, to a disorder with a better survival rate, creating a window for atrioventricular valve ...
Eva C. van der Leest   +12 more
wiley   +1 more source

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