Osteogenesis imperfecta

A Forlino, JC Marini - The Lancet, 2016 - thelancet.com
Osteogenesis imperfecta is a phenotypically and molecularly heterogeneous group of
inherited connective tissue disorders that share similar skeletal abnormalities causing bone …

New perspectives on osteogenesis imperfecta

A Forlino, WA Cabral, AM Barnes… - Nature Reviews …, 2011 - nature.com
A new paradigm has emerged for osteogenesis imperfecta as a collagen-related disorder.
The more prevalent autosomal dominant forms of osteogenesis imperfecta are caused by …

[HTML][HTML] Voluntary rewards mediate the evolution of pool punishment for maintaining public goods in large populations

T Sasaki, S Uchida, X Chen - Scientific reports, 2015 - nature.com
Punishment is a popular tool when governing commons in situations where free riders
would otherwise take over. It is well known that sanctioning systems, such as the police and …

Collagens—structure, function, and biosynthesis

K Gelse, E Pöschl, T Aigner - Advanced drug delivery reviews, 2003 - Elsevier
The extracellular matrix represents a complex alloy of variable members of diverse protein
families defining structural integrity and various physiological functions. The most abundant …

[PDF][PDF] The use of whole organ decellularization for the generation of a vascularized liver organoid

PM Baptista, MM Siddiqui, G Lozier, SR Rodriguez… - …, 2011 - Wiley Online Library
A major roadblock to successful organ bioengineering is the need for a functional vascular
network within the engineered tissue. Here, we describe the fabrication of three …

Extracellular matrix scaffolding in angiogenesis and capillary homeostasis

M Marchand, C Monnot, L Muller, S Germain - Seminars in cell & …, 2019 - Elsevier
The extracellular matrix (ECM) of blood vessels, which is composed of both the vascular
basement membrane (BM) and the interstitial ECM is identified as a crucial component of …

[HTML][HTML] Evidence that fibroblasts derive from epithelium during tissue fibrosis

M Iwano, D Plieth, TM Danoff, C Xue… - The Journal of …, 2002 - Am Soc Clin Investig
Interstitial fibroblasts are principal effector cells of organ fibrosis in kidneys, lungs, and liver.
While some view fibroblasts in adult tissues as nothing more than primitive mesenchymal …

Endothelial extracellular matrix: biosynthesis, remodeling, and functions during vascular morphogenesis and neovessel stabilization

GE Davis, DR Senger - Circulation research, 2005 - Am Heart Assoc
The extracellular matrix (ECM) is critical for all aspects of vascular biology. In concert with
supporting cells, endothelial cells (ECs) assemble a laminin-rich basement membrane …

Collagens, modifying enzymes and their mutations in humans, flies and worms

J Myllyharju, KI Kivirikko - TRENDS in Genetics, 2004 - cell.com
Collagens and proteins with collagen-like domains form large superfamilies in various
species, and the numbers of known family members are increasing constantly. Vertebrates …

Serpin structure, mechanism, and function

PGW Gettins - Chemical reviews, 2002 - ACS Publications
Serpins are a superfamily of proteins, whose membership is based on the presence of a
single common core domain consisting of three β-sheets and 8-9 R-helices, and with a set of …