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 …

Williams syndrome

BA Kozel, B Barak, CA Kim, CB Mervis… - Nature Reviews …, 2021 - nature.com
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 …

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 …

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 …

A generative modeling framework for inferring families of biomechanical constitutive laws in data-sparse regimes

M Yin, Z Zou, E Zhang, C Cavinato… - Journal of the …, 2023 - Elsevier
Quantifying biomechanical properties of the human vasculature could deepen our
understanding of cardiovascular diseases. Standard nonlinear regression in constitutive …

JAGGED1/NOTCH3 activation promotes aortic hypermuscularization and stenosis in elastin deficiency

JM Dave, R Chakraborty, A Ntokou… - The Journal of …, 2022 - Am Soc Clin Investig
Obstructive arterial diseases, including supravalvular aortic stenosis (SVAS),
atherosclerosis, and restenosis, share 2 important features: an abnormal or disrupted elastic …

Elastin-driven genetic diseases

MLD Lasio, BA Kozel - Matrix Biology, 2018 - Elsevier
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 …

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 …

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 …

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 …