We have identified the Drosophila UDP-glucose dehydrogenase gene as being involved in wingless signaling. Mutations in this gene, called kiwi, generate a phenotype identical to that of wingless. UDP-glucose dehydrogenase is required for the biosynthesis of UDP-glucuronate, which in turn is utilized in the biosynthesis of glycosaminoglycans. By rescuing the kiwi phenotype with both UDP-glucurona...

A considerable need exists for improved biomarkers for differential diagnosis, prognosis and monitoring of therapeutic interventions for mucopolysaccharidoses (MPS), inherited metabolic disorders that involve lysosomal storage of glycosaminoglycans. Here we report a simple, reliable method based on the detection of abundant nonreducing ends of the glycosaminoglycans that accumulate in cells, bl...

Low molecular weight heparin (LMWH) has a structure similar to heparan sulfate, which exerts anti‑inflammatory effects via inhibiting elastase (Ela) activity. Release of Ela along the glomerular capillary wall may induce glomerular injury and proteinuria. The present study aimed to investigate the influence of LMWH on steroid‑sensitive nephrotic syndrome (SSNS) and the potential underlying mech...

Glycosaminoglycans (GAGs) are complex carbohydrates that are ubiquitously present on the cell surface and in the extracellular matrix. Interactions between GAGs and pathogens represent the first line of contact between pathogen and host cell and are crucial to a pathogen's invasive potential. Their complexity and structural diversity allow GAGs to control a wide array of biological interactions...

The sugar components of acidic glycosaminoglycans have long been known to be derived from glucose without alteration of the sugar ring (Topper & Lipton, 1953). The discovery of the nucleotide sugars by Caputto et al. (1950) and Park (1952) radically transformed the outlook on the biosynthesis of polysaccharides. It became rapidly apparent, not only that nucleotide sugars were the ‘activated’ do...

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Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B leading to the accumulation of dermatan sulfate. Birth prevalence is between 1 in 43,261 and 1 in 1,505,160 live births. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The characteristic skeletal dysp...

We have examined the role of cell surface glycosaminoglycans in fibronectin-mediated cell adhesion by analyzing the adhesive properties of Chinese hamster ovary cell mutants deficient in glycosaminoglycans. The results of our study suggest that the absence of glycosaminoglycans does not affect the initial attachment and subsequent spreading of these cells on substrata composed of intact fibrone...

The sulfo groups of glycosaminoglycans contribute to their high charge densities, and are critical for the role they play in various physiological and pathophysiological processes. Unfortunately, the sulfo groups can be hydrolyzed to inorganic sulfate. Thus, it is important to monitor the presence of these sulfo groups. In addition, free anions, including chloride, sulfate and acetate, are ofte...

The urothelium plays a pivotal role as a barrier between urine and its solutes and the underlying bladder. Bladder surface mucus is a critical component of this function. The biologic activity of mucus that imparts this barrier function is generated by the highly anionic polysaccharide components (eg, glycosaminoglycans), which are extremely hydrophilic and trap water at the outer layer of the ...