Defining the Extracellular Matrix of Rhabdomyosarcoma
Frontiers in Oncology, 2021 Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood with a propensity to metastasize. Current treatment for patients with RMS includes conventional systemic chemotherapy, radiation therapy, and surgical resection; nevertheless ...
Xiaolei Lian +17 more
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Aquaculture Reports, 2022 The razor clam, Sinonovacula constricta, must have acquired adaptive mechanisms to defense thermal pressure during evolution as it’s an eurythermal bivalve. However, the specific mechanisms remain unclear. To identify thermal responsive genes and unravel
Xianghui Kong +5 more
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Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions. [PDF]
PLoS ONE, 2012 The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template for elastic fibers. Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovascular and ocular symptoms, and tall stature.
Stuart A Cain +4 more
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LTBP-2 Has a Single High-Affinity Binding Site for FGF-2 and Blocks FGF-2-Induced Cell Proliferation. [PDF]
PLoS ONE, 2015 Latent transforming growth factor-beta-1 binding protein-2 (LTBP-2) belongs to the fibrillin-LTBP superfamily of extracellular matrix proteins. LTBPs and fibrillins are involved in the sequestration and storage of latent growth factors, particularly ...
Clementine Menz +6 more
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Transcriptome-Wide Expression Profiling in Skin Fibroblasts of Patients with Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. [PDF]
PLoS ONE, 2016 Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT), is likely the most common systemic heritable connective tissue disorder, and is mostly recognized by generalized joint hypermobility, joint instability complications ...
Nicola Chiarelli +7 more
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Transcriptome analysis of skin fibroblasts with dominant negative COL3A1 mutations provides molecular insights into the etiopathology of vascular Ehlers-Danlos syndrome. [PDF]
PLoS ONE, 2018 Vascular Ehlers-Danlos syndrome (vEDS) is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII), which is the major expressed collagen in blood vessels and hollow organs.
Nicola Chiarelli +4 more
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Colony formation in Phaeocystis antarctica: connecting molecular mechanisms with iron biogeochemistry [PDF]
Biogeosciences, 2018 Phaeocystis antarctica is an important phytoplankter of the Ross Sea where it dominates the early season bloom after sea ice retreat and is a major contributor to carbon export.
S. J. Bender +11 more
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Genes, 2021 Latent transforming growth factor β (TGFβ)-binding protein (LTBP) 4, a member of the LTBP family, shows structural homology with fibrillins. Both these protein types are characterized by calcium-binding epidermal growth factor-like repeats interspersed ...
Chi-Ting Su, Z. Urban
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Fibrillins 1 and 2 Perform Partially Overlapping Functions during Aortic Development*
Journal of Biological Chemistry, 2006 Fibrillin-rich microfibrils are extracellular assemblies that impart structural properties to the connective tissue. To elucidate the contribution of fibrillin-rich microfibrils to organogenesis, we have examined the vascular phenotype of a newly created
L. Carta +12 more
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