Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.4.99.6 (sialyltransferase)
 1,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Wiskott-Aldrich syndrome (WAS) is an X-linked recessive immunodeficiency affecting B lymphocytes, T lymphocytes, and platelets. Previous studies on lymphocytes from WAS patients have revealed that leu-kosialin (CD43), a cell-surface glycoprotein bearing approximately 90 O-linked oligosaccharide chains, shows an aberrant electrophoretic mobility. To determine whether this finding reflects a different pattern of O-linked glycosylation in WAS cells, we have compared healthy individuals and WAS patients with respect to glycosyltransferase activities in T lymphocytes, platelets, and Epstein-Barr virus (EBV)-immortalized B cell lines. Stimulation of peripheral T cells from normal individuals in vitro with anti-CD3 antibodies and interleukin-2 was associated with a 3-fold increase in UDP-GlcNAc:Gal beta 3GalNAc-R (GlcNAc to GalNAc) beta 6-N-acetylglucosaminyltransferase (core 2 GlcNAc-T) from 0.8 to 2.2 nmol/mg/h. In contrast, peripheral T lymphocytes from WAS patients showed an inversion of this phenotype with high core 2 GlcNAc-T activity in unstimulated cells (2.3 nmol/mg/h) and a 2-3-fold decrease in activity following stimulation. Core 2 GlcNAc-T activity was also three times higher in platelets from WAS patients than in normal platelets. Glycosyltransferase activities were measured in immortalized B cell lines established from WAS and normal subjects by infection with EBV. Core 2 GlcNAc-T was less than 0.4 nmol/mg/h in WAS EBV-B cell lines compared to 2.4 nmol/mg/h in EBV-B cell lines from healthy individuals, In contrast, CMP-SA:SA alpha 2-3Gal beta 1-3GalNAc-R (where SA represents sialyl (sialic acid to GalNAc) alpha 6-sialyltransferase II activity was 2.0 nmol/mg/h in the WAS EBV-B cell and less than .01 nmol/mg/h in EBV-B cell lines derived from normal subjects. Eleven other glycosyltransferase activities were measured and found to be similar in EBV-B cell lines from WAS and normal individuals. Polylactosamine sequences were much reduced in the O-linked oligosaccharides of CD43 from WAS EBV-B cells consistent with decreased core 2 GlcNAc-T activity and expression of core 1 oligosaccharides in the cells. In conclusion, B cells, T cells, and platelets in WAS patients show abnormal expression of two developmentally regulated glycosyltransferases, consistent with the idea that the WAS immunodeficiency is due to a failure of normal lymphocyte maturation.
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PMID:Aberrant O-linked oligosaccharide biosynthesis in lymphocytes and platelets from patients with the Wiskott-Aldrich syndrome. 200 80

Previous studies have demonstrated the ability of retinoic acid (RA) to inhibit the growth of two spontaneous murine melanoma cell lines (B16-F1 and S91-C2) and to augment both sialyltransferase activity and the sialylation of an Mr 160,000 cell-surface glycoprotein. The present study examined the effects of RA on an ultraviolet irradiation-induced murine melanoma cell line K-1735P. Like the two spontaneous melanomas, the uv-induced melanoma exhibited susceptibility to the growth-inhibitory action of RA. Both the anchorage-dependent and the anchorage-independent growths of the K-1735P cells were suppressed by RA, with IC50 values of 5 X 10(-9) and 3 X 10(-12) M, respectively. Sialyltransferase activity in both S91-C2 and K-1735P cells treated with 10(-6) or 10(-5) M RA increased two- and three-fold, respectively, as compared with untreated cells. In contrast, cell-surface sialo- and galactoglycoproteins, revealed by labeling with periodate and tritiated borohydrate or with neuraminidase, galactose oxidase, and tritiated borohydrate, respectively, varied between the S91-C2 and the K-1735P cells, and each cell line's modulation by RA was also distinct. These findings suggest that although RA can increase the activity of sialyltransferase in different melanoma cells, this increased activity may, in turn, result in an increased sialylation of distinct cell-surface glycoproteins.
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PMID:Enhancement of sialyltransferase in two melanoma cell lines that are growth-inhibited by retinoic acid results in increased sialylation of different cell-surface glycoproteins. 339 Dec 45