Elastin, arterial mechanics, and cardiovascular disease
AJ Cocciolone, JZ Hawes… - American Journal …, 2018 - journals.physiology.org
Large, elastic arteries are composed of cells and a specialized extracellular matrix that
provides reversible elasticity and strength. Elastin is the matrix protein responsible for this …
provides reversible elasticity and strength. Elastin is the matrix protein responsible for this …
Williams syndrome
Williams syndrome (WS) is a relatively rare microdeletion disorder that occurs in as many as
1: 7,500 individuals. WS arises due to the mispairing of low-copy DNA repetitive elements at …
1: 7,500 individuals. WS arises due to the mispairing of low-copy DNA repetitive elements at …
Elastic fibers and biomechanics of the aorta: Insights from mouse studies
H Yanagisawa, J Wagenseil - Matrix Biology, 2020 - Elsevier
Elastic fibers are major components of the extracellular matrix (ECM) in the aorta and
support a life-long cycling of stretch and recoil. Elastic fibers are formed from mid-gestation …
support a life-long cycling of stretch and recoil. Elastic fibers are formed from mid-gestation …
Cardiovascular disease in Williams syndrome
II R Thomas Collins - Current opinion in pediatrics, 2018 - journals.lww.com
Cardiovascular abnormalities are a major determining factor in the clinical picture and
trajectory of patients with Williams syndrome. Advances in surgical techniques, medical …
trajectory of patients with Williams syndrome. Advances in surgical techniques, medical …
A generative modeling framework for inferring families of biomechanical constitutive laws in data-sparse regimes
Quantifying biomechanical properties of the human vasculature could deepen our
understanding of cardiovascular diseases. Standard nonlinear regression in constitutive …
understanding of cardiovascular diseases. Standard nonlinear regression in constitutive …
JAGGED1/NOTCH3 activation promotes aortic hypermuscularization and stenosis in elastin deficiency
Obstructive arterial diseases, including supravalvular aortic stenosis (SVAS),
atherosclerosis, and restenosis, share 2 important features: an abnormal or disrupted elastic …
atherosclerosis, and restenosis, share 2 important features: an abnormal or disrupted elastic …
Elastin-driven genetic diseases
Elastic fibers provide recoil to tissues that undergo repeated deformation, such as blood
vessels, lungs and skin. Composed of elastin and its accessory proteins, the fibers are …
vessels, lungs and skin. Composed of elastin and its accessory proteins, the fibers are …
Vascular development
MW Majesky - Arteriosclerosis, thrombosis, and vascular biology, 2018 - Am Heart Assoc
The vascular system forms as a branching network of endothelial cells that acquire identity
as arterial, venous, hemogenic, or lymphatic. Endothelial specification depends on gene …
as arterial, venous, hemogenic, or lymphatic. Endothelial specification depends on gene …
Heterogeneous cellular contributions to elastic laminae formation in arterial wall development
CJ Lin, MC Staiculescu, JZ Hawes… - Circulation …, 2019 - Am Heart Assoc
Rationale: Elastin is an important ECM (extracellular matrix) protein in large and small
arteries. Vascular smooth muscle cells (SMCs) produce the layered elastic laminae found in …
arteries. Vascular smooth muscle cells (SMCs) produce the layered elastic laminae found in …
Central artery stiffness and thoracic aortopathy
JD Humphrey, G Tellides - American Journal of Physiology …, 2019 - journals.physiology.org
Thoracic aortopathy, especially aneurysm, dissection, and rupture, is responsible for
significant morbidity and mortality. Uncontrolled hypertension and aging are primary risk …
significant morbidity and mortality. Uncontrolled hypertension and aging are primary risk …