Qualitative and quantitative determinations of glycosaminoglycans in the nonswelling corneal stroma of the spiny dogfish shark, Squalus acanthias, were made with the use of a cetylpyridinium chloride-cellulose microfractionation technique. Dogfish stroma was found to contain predominantly highly sulfated galactosaminoglycans forming approximately 75 per cent of the total glycosaminoglycans when...

The relative binding affinity of Zn2+ to several glycosaminoglycans was determined by gel-filtration chromatography. Binding was observed only between Zn2+ and heparin. No binding was observed between Zn2+ and chondroitin 4-sulphate, chondroitin 6-sulphate, dermatan sulphate or hyaluronic acid. All of the glycosaminoglycans contained carboxy groups, but only heparin bound Zn2+. This observation...

A large amount of glycosaminoglycans was found in the AH-130 ascites tumor cells and also in the ascites fluids. After combined administration of Cyclophosphamide and vitamin A to the tumor-bearing rats, a significant decrease was found in tumor glycosaminoglycans sensitive to lysosomal hyaluronidase, such as the nonsulfated glycosamino glycans or chondroitin sulfate A and/or C. An increased re...

This direct method for quantifying excessive urinary glycosaminoglycan excretion exploits the specific binding of 1,9-dimethylmethylene blue (DMB). The procedure obviates cumbersome and labor-intensive procedures for separating glycosaminoglycans from other constituents of urine. Pediatric pharmaceutical formulations (except heparin), in concentrations expected in urine, do not interfere with s...

OBJECTIVE To describe the profile of joint mobility and grip and pinch strength of MPS VI patients and to correlate this with urinary excretion of glycosaminoglycans (GAGs), ARSB activity, and the distance covered in a 6-minute walking test (6MWT). METHODS This was an observational study of 28 patients with MPS VI, who had not undergone specific treatment. All patients were assessed for ampli...

The mucopolysaccharidoses (MPSs) often present a diagnostic challenge, particularly for patients who have more slowly progressive disease phenotypes, as early disease manifestations can be subtle or non-specific. However, certain types of bone and joint involvement should always prompt consideration of an MPS diagnosis, such as early joint involvement without classic inflammatory features or er...

Glycosaminoglycans (GAGs) have been identified as one of the macromolecules present in the stone matrix. Although electrophoresis has been used to analyze the composition of GAGs, this method is laborious and insensitive. The aim of this study was to examine GAG profiles in the stone matrix from 4 types of stones using high-performance liquid chromatography (HPLC). This method has been shown to...

In the normal mouse embryo, Bmp4 is expressed in mesenchymal cells surrounding the Wolffian duct (WD) and ureter stalk, whereas bone morphogenetic protein (BMP) type I receptor genes are transcribed either ubiquitously (Alk3) or exclusively in the WD and ureter epithelium (Alk6). Bmp4 heterozygous null mutant mice display, with high penetrance, abnormalities that mimic human congenital anomalie...

The detachment forces of various polymers are frequently measured to determine their mucoadhesion strength. As the process of mucoadhesion is a consequence of interactions between the mucus layer on mucosa and mucoadhesive polymers, it is greatly dependent on mucus and polymer structure including their charge. It is also known that the glycosaminoglycan layer, which covers the urinary bladder m...