Results 31 to 40 of about 16,351 (268)

Expanding genetic and clinical aspects of Schwartz-Jampel syndrome: A report of two cases with literature review [PDF]

open access: yesMolecular Genetics and Metabolism Reports
Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by muscle stiffness (myotonia) and chondrodysplasia. This disease is caused by biallelic loss of function mutations in the HSPG2 gene, which encodes the core protein of ...
Iman Elahi Vahed   +7 more
doaj   +2 more sources

Life without Perlecan Has Its Problems [PDF]

open access: bronzeThe Journal of Cell Biology, 1999
Perlecan is a large heparan sulfate proteoglycan with a wide tissue distribution and multiple potential functions (see [Iozzo 1998][1]). The glycosaminoglycan chains, located in the NH2-terminal domain of the core protein, bind basic FGF-2 and have been shown to promote the mitogenic and ...
Bjørn R. Olsen
openalex   +4 more sources

Developmental expression of perlecan during murine embryogenesis [PDF]

open access: bronzeDevelopmental Dynamics, 1997
Perlecan is a modular heparan sulfate proteoglycan that is an intrinsic constituent of all basement membranes and extracellular matrices. Because of its strategic position and unique structure, perlecan has been implicated in modulating the activity of various growth factors required for normal development and tissue homeostasis.
Melissa Handler   +2 more
openalex   +4 more sources

The Fbn1 gene variant governs passive ascending aortic mechanics in the mgΔlpn mouse model of Marfan syndrome when superimposed to perlecan haploinsufficiency [PDF]

open access: yesFrontiers in Cardiovascular Medicine
IntroductionAscending thoracic aortic aneurysms arise from pathological tissue remodeling that leads to abnormal wall dilation and increases the risk of fatal dissection/rupture. Large variability in disease manifestations across family members who carry
Samar A. Tarraf   +4 more
doaj   +2 more sources

Recombinant perlecan domain V covalently immobilized on silk biomaterials via plasma immersion ion implantation supports the formation of functional endothelium.

open access: yesJournal of Biomedical Materials Research. Part A, 2023
Strategies to promote rapid formation of functional endothelium are required to maintain blood fluidity and regulate smooth muscle cell proliferation in synthetic vascular conduits.
K. Lau   +7 more
semanticscholar   +1 more source

Lead suppresses perlecan expression via EGFR-ERK1/2-COX-2-PGI2 pathway in cultured bovine vascular endothelial cells.

open access: yesJournal of Toxicological Sciences, 2023
Vascular endothelial cell growth is essential for the repair of intimal injury. Perlecan, a large heparan sulfate proteoglycan, intensifies fibroblast growth factor-2 (FGF-2) signaling as a co-receptor for FGF-2 and its receptor, and promotes the ...
Takato Hara   +7 more
semanticscholar   +1 more source

Regulation of Glycoprotein VI-Dependent Platelet Activation and Thrombus Formation by Heparan Sulfate Proteoglycan Perlecan

open access: yesInternational Journal of Molecular Sciences, 2023
Proteoglycans form a heterogeneous family of proteins with covalently bound sulfated glycosaminoglycans. The extracellular matrix proteoglycan perlecan has been proposed to bind to the platelet- and megakaryocyte-specific receptor G6bB, co-regulating ...
Isabella Provenzale   +5 more
semanticscholar   +1 more source

Novel interactions of perlecan: Unraveling perlecan's role in angiogenesis [PDF]

open access: yesMicroscopy Research and Technique, 2008
AbstractPerlecan, a highly conserved and ubiquitous basement membrane heparan sulfate proteoglycan, is essential for life, inasmuch as its absence results in embryonic lethality in mice and C. elegans, and neonatal lethality in humans. Perlecan plays an essential role in vasculogenesis and chondrogenesis, as well as in pathological states where these ...
Gregory J. Bix, Renato V. Iozzo
openaire   +3 more sources

Perlecan and Tumor Angiogenesis [PDF]

open access: yesJournal of Histochemistry & Cytochemistry, 2003
Perlecan is a major heparan sulfate proteoglycan (HSPG) of basement membranes (BMs) and connective tissues. The core protein of perlecan is divided into five domains based on sequence homology to other known proteins. Commonly, the N-terminal domain I of mammalian perlecan is substituted with three HS chains that can bind a number of matrix molecules,
Jiang, Xinnong, Couchman, John R
openaire   +3 more sources

Perlecan is critical for heart stability [PDF]

open access: yesCardiovascular Research, 2008
Perlecan is a heparansulfate proteoglycan found in basement membranes, cartilage, and several mesenchymal tissues that form during development, tumour growth, and tissue repair. Loss-of-function mutations in the perlecan gene in mice are associated with embryonic lethality caused primarily by cardiac abnormalities probably due to hemopericards. The aim
Yun Fan   +17 more
openaire   +3 more sources

Home - About - Disclaimer - Privacy