Search results for "Glycan"

Safety study of sodium pentosan polysulfate for adult patients with mucopolysaccharidosis type II

2019

Current therapies for the mucopolysaccharidoses (MPS) do not effectively address skeletal and neurological manifestations. Pentosan polysulfate (PPS) is an alternative treatment strategy that has been shown to improve bone architecture, mobility, and neuroinflammation in MPS animals. The aims of this study were to a) primarily establish the safety of weekly PPS injections in attenuated MPS II, b) assess the efficacy of treatment on MPS pathology, and c) define appropriate clinical endpoints and biomarkers for future clinical trials. Subcutaneous injections were administered to three male Japanese patients for 12 weeks. Enzyme replacement therapy was continued in two of the patients while th…

Possible control mechanism of cell motility in the gorgonian Eunicella cavolinii

1985

In a previous study it was demonstrated that a lectin controls cell-cell interaction in the gorgonian Eunicella cavolinii (Koch) as a negative modulator. Now we describe the procedure to purify this lectin to homogeneity; its molecular weight is 23 400. The homologous proteoglycans were identified as positive modulators of cell-cell (and/or cell substrate) interaction. The purified single proteoglycan aggregates were 1200±700 nm long and the distance between the attachment points of the proteoglycan subunits was about 45 nm. The glycosaminoglycan residues of the gorgonian proteoglycans were identified as hyaluronic acid (35.5%), heparan sulfate (47.9%) and dermatan sulfate (14.1%). Binding …

Fetal presentation of Morquio disease type A.

1992

A fetus with mucopolysaccharidosis type IV A (Morquio type A) is described. The family had one affected child exhibiting symptoms of classical Morquio A disease, and late in the subsequent pregnancy prenatal diagnosis was requested. At 23 weeks' gestation, moderate ascites was detected by detailed ultrasound scan and keratan sulphate was found in the amniotic fluid. The pregnancy was terminated by prostaglandin induction and the diagnosis of mucopolysaccharidosis type IV A was confirmed by demonstration of a deficiency of N-acetylgalactosamine-6-sulphate (GalNac-6-S) sulphatase in cultured amniotic cells and in post-mortem fibroblast cultures. The activities of beta-galactosidase and arylsu…

Disruption of apical-basal polarity of human embryonic stem cells enhances hematoendothelial differentiation

2007

Abstract During murine development, the formation of tight junctions and acquisition of polarity are associated with allocation of the blastomeres on the outer surface of the embryo to the trophoblast lineage, whereas the absence of polarization directs cells to the inner cell mass. Here, we report the results of ultrastructural analyses that suggest a similar link between polarization and cell fate in human embryos. In contrast, the five human embryonic stem cell (hESC) lines displayed apical-basal, epithelial-type polarity with electron-dense tight junctions, apical microvilli, and asymmetric distribution of organelles. Consistent with these findings, molecules that are components of tigh…

Expression patterns of matrix genes during human skeletal development.

1994

Characterization of PAMP/PRR interactions in European eel (Anguilla anguilla) macrophage-like primary cell cultures

2013

The eel (Anguilla anguilla) has been identified as a vulnerable species with stocks dramatically declining over the past decade. In an effort to support the species from overfishing of wild stocks increased interest in eel aquaculture has been notable. In order to expand the scarce knowledge concerning the biology of this species significant research efforts are required in several fields of biology. The development of cell culture systems to study the immune response is a key step towards an increased understanding of the immune response and to develop resources to support further study in this threatened species. Macrophages are one of the most important effector cells of the innate immun…

THE ROLE OF INTRAVESICAL GLYCOSAMINOGLYCANS IN TOXICITY INDUCED BY ADJUVANT INTRAVESICAL THERAPY: GENETIC LABORATORY EVIDENCE

2015

Analytical technique for studying the structure of glycoprotein N-glycans

1993

Abstract The aim of this study was to develop an analytical strategy for the structural analysis of glycoprotein N-glycans by combining several sensitive methods without any elaborate equipment. The following consecutive steps were optimized and applied: (1) immobilization of glycopeptide N-glycosidase F (EC 3.2.2.18) on several polymeric and a silica support, the latter giving a maximum binding capacity of 11.3% of starting activity; (2) lectin affinity chromatography was miniaturized using Mobitec columns of volume 200 μl; the binding capacity of glycoproteins on concanavalin A and wheat germ agglutinin columns was in the range 0.5–1 μg; (3) N-linked oligosaccharides were isolated from co…

Glycosylation deficiency at either one of the two glycan attachment sites of cellular prion protein preserves susceptibility to bovine spongiform enc…

2004

The conversion into abnormally folded prion protein (PrP) plays a key role in prion diseases. PrP(C) carries two N-linked glycan chains at amino acid residues 180 and 196 (mouse). Previous in vitro data indicated that the conversion process may not require glycosylation of PrP. However, it is conceivable that these glycans function as intermolecular binding sites during the de novo infection of cells on susceptible organisms and/or play a role for the interaction of both PrP isoforms. Such receptor-like properties could contribute to the formation of specific prion strains. However, in earlier studies, mutations at the glycosylation sites of PrP led to intracellular trafficking abnormalitie…

A BMP7 Variant Inhibits Tumor Angiogenesis In Vitro and In Vivo through Direct Modulation of Endothelial Cell Biology

2015

Bone morphogenetic proteins (BMPs), members of the TGF-β superfamily, have numerous biological activities including control of growth, differentiation, and vascular development. Using an in vitro co-culture endothelial cord formation assay, we investigated the role of a BMP7 variant (BMP7v) in VEGF, bFGF, and tumor-driven angiogenesis. BMP7v treatment led to disruption of neo-endothelial cord formation and regression of existing VEGF and bFGF cords in vitro. Using a series of tumor cell models capable of driving angiogenesis in vitro, BMP7v treatment completely blocked cord formation. Pre-treatment of endothelial cells with BMP7v significantly reduced their cord forming ability, indicating …