Band 3 and PAS-1 (a dimer of glycophorin A) from erythrocyte membranes of three children with congenital disorder of glycosylation, type Ia (CDG-Ia), aged 1 month, 3 years and 10 years respectively, were examined by a new technique that allowed determination of carbohydrate molar composition of glycoproteins separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis. In CDG children...

PURPOSE The purpose of this study was to determine whether plasma biomarkers reflect changes in lung function and respiratory exacerbations associated with CF lung disease. METHODS Plasma human leukocyte elastase/alpha1 antitrypsin complex (pHLE complex) values were measured in 28 adult CF patients and 47 healthy volunteers and correlated with forced expiratory volume (FEV1) and forced vital ...

The effect of strong electrolyte upon the activity of Vibrio cholerae neuraminidase (VC neuraminidase) toward the surface of intact cells and toward their isolated, purified sialolipid components, was studied in detail. A linear dependence of the logarithm of initial velocity on the square root of ionic strength was observed for purified GM8 over a concentration range of 0 to 0.05 M NaCl in 0.0...

Sialylation is essential for development and regeneration in mammals. Using N-propanoylmannosamine, a novel precursor of sialic acid, we were able to incorporate unnatural sialic acids with a prolonged N-acyl side chain (e.g., N-propanoylneuraminic acid) into cell surface glycoconjugates. Here we report that this biochemical engineering of sialic acid leads to a stimulation of neuronal cells. B...

BACKGROUND Sialic acid (N-acetylneuraminic acid), a component of gangliosides and sialylglycoproteins, may be a conditional nutrient in early life because endogenous synthesis is limited. The aim of this study was to investigate the metabolic fate of intravenously administrated N-acetylneuraminic acid-6-14C (sialic acid) in piglets. METHOD Three-day-old male domestic piglets (Sus scrofa) were...

Two sialic acids were isolated from the jelly coat of the eggs of the sea urchin Pseudocentrotus depressus. Both were subjected to chemical assay, thin layer chromatography, infrared absorption study, and enzymatic treatment. The results indicate that one of the sialic acids is identical with N-glycolylneuraminic acid. The other sialic acid is a completely new type and was deduced to be N-aceto...

Previous studies have shown that Sindbis virus, an enveloped alphavirus of the togavirus group, activates the alternative complement pathway in the absence of detectable antiviral immunoglobulin. The present studies examined the role of the host-determined sialic acid content of Sindbis virus on activation of the alternative complement pathway. Purified Sindbis virus grown in baby hamster kidne...

Monomer proteoglycan was isolated from porcine ovarian follicular fluid by isopycnic CsCl centrifugation in the presence of 4 M guanidine HCl and protease inhibitors. The elution profile of the D1 preparation on Sepharose 2B was similar to that of monomer proteoglycan from bovine nasal cartilage, indicating a similar molecular size. Follicular fluid proteoglycans consist of about 20% protein, 5...

We have previously engineered transgenic insect cell lines to express mammalian glycosyltransferases and showed that these cells can sialylate N-glycoproteins, despite the fact that they have little intracellular sialic acid and no detectable CMP-sialic acid. In the accompanying study, we presented evidence that these cell lines can salvage sialic acids for de novo glycoprotein sialylation from...

Sialic acids are a family of amino sugars that are commonly found as terminal oligosaccharide residues on glycoproteins and glycolipids. Plasma lipoproteins are sialylated on their apolipoprotein and glycolipid constituents. The function of sialic acid on apolipoproteins is not completely understood but has been associated with secretion, lipid-binding, and plasma clearance for some apolipoprot...