[HTML][HTML] Elastic fibers during aging and disease

A Heinz - Ageing research reviews, 2021 - Elsevier
Elastic fibers are essential constituents of the extracellular matrix of higher vertebrates and
endow several tissues and organs including lungs, skin and blood vessels with elasticity …

TGF-β and BMP signaling pathways in skeletal dysplasia with short and tall stature

A Costantini, A Guasto… - Annual Review of …, 2023 - annualreviews.org
The transforming growth factor β (TGF-β) and bone morphogenetic protein (BMP) signaling
pathways play a pivotal role in bone development and skeletal health. More than 30 different …

Multi-ancestry genome-wide association analyses improve resolution of genes and pathways influencing lung function and chronic obstructive pulmonary disease risk

N Shrine, AG Izquierdo, J Chen, R Packer, RJ Hall… - Nature …, 2023 - nature.com
Lung-function impairment underlies chronic obstructive pulmonary disease (COPD) and
predicts mortality. In the largest multi-ancestry genome-wide association meta-analysis of …

ADAMTS proteases in cardiovascular physiology and disease

S Santamaria, R de Groot - Open biology, 2020 - royalsocietypublishing.org
The a disintegrin-like and metalloproteinase with thrombospondin motif (ADAMTS) family
comprises 19 proteases that regulate the structure and function of extracellular proteins in …

[HTML][HTML] The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly

ARF Godwin, M Singh, MP Lockhart-Cairns, YF Alanazi… - Matrix Biology, 2019 - Elsevier
Fibrillin is a large evolutionarily ancient extracellular glycoprotein that assembles to form
beaded microfibrils which are essential components of most extracellular matrices. Fibrillin …

Regulation of ADAMTS proteases

KWJ Rose, N Taye, SZ Karoulias… - Frontiers in Molecular …, 2021 - frontiersin.org
A disintegrin and metalloprotease with thrombospondin type I motifs (ADAMTS) proteases
are secreted metalloproteinases that play key roles in the formation, homeostasis and …

ADAMTS10 inhibits aggressiveness via JAK/STAT/c-MYC pathway and reprograms macrophage to create an anti-malignant microenvironment in gastric cancer

J Zhou, T Li, H Chen, Y Jiang, Y Zhao, J Huang… - Gastric Cancer, 2022 - Springer
Background A disintegrin and metalloproteinase with thrombospondin motifs 10
(ADAMTS10) plays a role in extracellular matrix and correlates with Weill–Marchesani …

Acromelic dysplasias: how rare musculoskeletal disorders reveal biological functions of extracellular matrix proteins

S Stanley, Z Balic, D Hubmacher - … of the new York Academy of …, 2021 - Wiley Online Library
Acromelic dysplasias are a group of rare musculoskeletal disorders that collectively present
with short stature, pseudomuscular build, stiff joints, and tight skin. Acromelic dysplasias are …

Zinn's zonule

S Bassnett - Progress in retinal and eye research, 2021 - Elsevier
Abstract The Zonule of Zinn, or ciliary zonule, is the elaborate system of extracellular fibers
that centers the lens in the eye. In humans, the fibers transmit forces that flatten the lens …

A novel ADAMTS17 variant that causes Weill-Marchesani syndrome 4 alters fibrillin-1 and collagen type I deposition in the extracellular matrix

SZ Karoulias, A Beyens, Z Balic, S Symoens… - Matrix Biology, 2020 - Elsevier
Weill-Marchesani syndrome (WMS) is a rare genetic disorder that affects the
musculoskeletal system, the eye, and the cardiovascular system. Individuals with WMS …