Abstract:
:Enzyme replacement therapy (ERT) is the worldwide standard of care for a number of mucopolysaccharidosis (MPS) diseases. We report a kinetic study of plasmatic dermatan sulfate (DS) in a 3-year-old subject affected by a severe form of MPS II during the first 10 months of ERT with Idursulfase. A strong increase in the DS plasmatic concentration was measured immediately after the first enzyme infusion, with a maximum after 3 h, followed by a continuous decrease in the 8-15 days following the beginning of treatment. After this, a constant plasmatic content of DS concentration was observed. Overall, during the 10-month treatment period, ERT reduced the plasmatic concentration of DS up to ~80-85 %, but it was unable to totally remove it from the blood. We can suppose that immediately after the first enzyme administrations, a large amount of abnormal DS is removed from tissues reaching the blood compartment and eliminated via the urine, and thereafter only minimal changes are observed. The persistency of the residual amounts of DS with the actually recommended dosage in our Patient may suggest the opportunity to promote further studies with increased enzyme dosages to completely remove the accumulation of lysosomal DS.
journal_name
Glycoconj Jjournal_title
Glycoconjugate journalauthors
Volpi N,Zampini L,Maccari F,Santoro L,Galeotti F,Galeazzi T,Gabrielli O,Coppa GVdoi
10.1007/s10719-013-9471-0subject
Has Abstractpub_date
2013-10-01 00:00:00pages
727-32issue
7eissn
0282-0080issn
1573-4986journal_volume
30pub_type
杂志文章abstract::A sialoglycopeptide was isolated from buffalo colostrum in pure form by chromatography on Sephadex G-25 and QAE-Sephadex A-25. This was found to be homogeneous by cellulose acetate membrane electrophoresis and reverse phase HPLC. It consisted of fucose, galactose, mannose, N-acetyl glucosamine and N-acetyl neuraminic ...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF01049681
更新日期:1996-02-01 00:00:00
abstract::To elucidate the biological significance of the lactosylceramide (LacCer) branching in glycosphingolipid (GSL) biosynthesis, we established ganglioside GM3- and lactosylsulfatide SM3-reconstituted cells by introducing the GM3 synthase gene and the sulfotransferase gene, respectively. In SM3-expressing cells, the reduc...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/B:GLYC.0000024248.62242.1f
更新日期:2004-01-01 00:00:00
abstract::The sugar chain-binding specificity of tomato lectin (LEA) against glycoproteins was investigated qualitatively using lectin blot analysis. Glycoproteins containing tri- and tetra-antennary complex-type N-glycans were stained with LEA. Unexpectedly, glycoproteins containing high mannose-type N-glycans and a horseradis...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-005-5329-4
更新日期:2005-11-01 00:00:00
abstract::Neutrophils participate in host protection and central to this process is the regulation of oxidative mechanisms. We purified by affinity chromatography the receptor for the GlcNAc-specific WGA from CD14+ CD16+ cell lysates (WGAr). The receptor is a 141 kDa glycoprotein constituted by two subunits of 78 and 63 kDa. It...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-006-8635-6
更新日期:2006-11-01 00:00:00
abstract::Human β-1,4-galactosyltransferase (β-1,4-GalT) V was shown to be involved in the biosynthesis of N-glycans, O-glycans and lactosylceramide (Lac-Cer) by in vitro studies. To determine its substrate specificity, enzymatic activity and its products were analyzed using mouse embryonic fibroblast (MEF) cells from β-1,4-Gal...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-010-9313-2
更新日期:2010-10-01 00:00:00
abstract::We have measured glycosyltransferase activities of SupT1 cells, a T-lymphoid cell line shown to react with autoantibodies in the sera of many HIV patients. Since considerable alpha-N-acetylgalactosaminyl-transferase and beta 1, 3 galactosyltransferase activities were found in SupT1 cells, at least the O-glycan core 1 ...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731338
更新日期:1995-06-01 00:00:00
abstract::The main component of the ganglioside(1) mixture from the brain of the adult amphibian Xenopus laevis accounts for 35% of the total, as lipid bound sialic acid. This ganglioside has been purified and characterized by thin layer chromatography, partial and exhaustive enzymatic hydrolysis with sialidase, TLC-overlay pro...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1022584914778
更新日期:2002-01-01 00:00:00
abstract::Alterations in cell membrane glycosylation play important role in oral carcinogenesis. The present study evaluated salivary sialylation changes i.e. total sialic acid (TSA), sialidase activity, linkage specific (α2-3 and α2-6) sialoproteins and sialyl transferase (ST) activity in controls, patients with oral precancer...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-014-9561-7
更新日期:2014-12-01 00:00:00
abstract::The human glioma D-54MG cell line grown in vitro primarily expresses ganglio series gangliosides, particularly GM2. Subcutaneous injection of these cells into nude mice produced xenografts with an increased content of the human glioma-associated lacto series gangliosides, primarily 3'-isoLM1, an alteration that was do...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731472
更新日期:1996-06-01 00:00:00
abstract::In this study the comparative TLC immunostaining investigation of neutral GSLs and gangliosides from human skeletal and heart muscle is described. A panel of specific polyclonal and monoclonal antibodies as well as the GM1-specific choleragenoid were used for the overlay assays, combined with preceding neuraminidase t...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1018552729572
更新日期:1997-01-01 00:00:00
abstract::Both infiltrating leukocytes and soluble immunoglobulin form aggregates in synovial fluid during the inflammatory process in rheumatoid arthritis (RA). Some of these changes are probably mediated by the adhesion molecule, E-selectin, which increases its expression with disease activity. As glycosylation changes in IgG...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1006920007227
更新日期:1998-12-01 00:00:00
abstract::The Maillard reaction was discovered in 1912 by Louis C. Maillard when he observed the browning phenomena with aroma formation in a heated solution containing a sugar and an amino acid. The Maillard reaction starts from the reactions between carbonyl groups of various sugars and amino groups of amino acids/ proteins, ...
journal_title:Glycoconjugate journal
pub_type: 杂志文章,评审
doi:10.1007/s10719-020-09943-x
更新日期:2020-09-10 00:00:00
abstract::Synthesis and antigenic reactivity of 6-O-sulfo sialylparagloboside (SPG) and sialyl Lewis X (sLeX) neo-glycolipids containing lactamized neuraminic acid are described. The suitably protected GlcNAc-beta (1-->3)-Gal-beta (1-->4)-GlcOSE derivative was glycosylated with NeuTFAc-alpha (2-->3)-Gal imidate to give NeuTFAc-...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-005-0352-z
更新日期:2005-03-01 00:00:00
abstract::Galectins are animal lectins that can specifically bind beta-galactosides. Thirteen galectins have already been described. This review focuses on a specific member of this family: galectin-8. This galectin was discovered in prostate cancer cells eight years ago and has been studied extensively in the last few years. T...
journal_title:Glycoconjugate journal
pub_type: 杂志文章,评审
doi:10.1023/B:GLYC.0000014086.38343.98
更新日期:2002-01-01 00:00:00
abstract::Twenty different human and animal tissues were investigated for the presence of polyglycosylceramides. The glycolipids were isolated by peracetylation of dry tissue residues left after conventional lipid extraction, followed by extraction with chloroform and subsequent Sephadex LH-20, Sephadex LH-60 and silica gel chr...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1018545922728
更新日期:1997-02-01 00:00:00
abstract::Homology searches indicated that up to five class I α-mannosidases (glycohydrolase family 47) and eight class II α-mannosidases (glycohydrolase family 38) are encoded by the fruitfly (Drosophila melanogaster) genome. Selected example mannosidases were expressed in secreted form using the yeast Pichia pastoris. A numbe...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-013-9495-5
更新日期:2013-12-01 00:00:00
abstract::Cultured chondrocytes derived from the caudal and cephalic ends of embryonic chick sterna have been compared with each other and with whole sternum, by using a panel of 21 lectins to probe the distribution of oligosaccharides in glycoconjugates of cells and matrix at various times of culture or development. On culture...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1018526813159
更新日期:1997-04-01 00:00:00
abstract::Heteropolysaccarides were isolated from the Korean medicinal plant, Phellodendri cortex (Hwangbek), by hot water and alkali extractions. The extracted polysaccharides were fractionated into eight fractions and they are mainly composed of D-N-acetylglucosamine, D-galactose, D-mannose, and D-glucose. Among the polysacch...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1007084506071
更新日期:1999-03-01 00:00:00
abstract::A non-enzymatic reaction between sugars or aldehydes and the amino groups of proteins, lipids and nucleic acids contributes to the aging of macromolecules, which could impair their structural integrity and function. This process begins with the conversion of reversible Schiff base adducts, and then to more stable, cov...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-016-9682-2
更新日期:2016-08-01 00:00:00
abstract::Matrix proteoglycans (PGs) have shown promise as biomarker in malignancies. We employed agarose gel eletrophoresis, quantitative real- time reverse transcription-polymerase chain reaction and immunohistochemistry to evaluate the content of sulfated glicosaminoglycans (chondroitin sulfate and heparan sulfate) and expre...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-018-9815-x
更新日期:2018-04-01 00:00:00
abstract::We have used two methods to evaluate the level of expression of Gb3Cer in several human leukaemia/lymphoma cell lines representative of the myeloid (K562, KG-1, HL-60, and lymphoid (Reh, Daudi, Raji, RPMI 8226, CCRF-CEM, MOLT-4) lineages blocked at varied stages of differentiation. TLC immunostaining of glycolipid ext...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731265
更新日期:1995-10-01 00:00:00
abstract::We analyzed surface glycoproteins of human natural killer (NK) cells by utilizing lectins. Among the lectins tested, wheat germ agglutinin (WGA) was found to bind preferentially to CD16(Leu11)-positive lymphocytes as determined by two-colour flow cytometry. Analysis of glycoproteins in the lysate prepared from NK cell...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731165
更新日期:1992-08-01 00:00:00
abstract::Potato lectin (Solanum tuberosum agglutinin, STA) is an unusual glycoprotein containing approximately 50% carbohydrates by weight. Of the total carbohydrates, 92% is contributed by L: -arabinose, which are O-linked to hydroxyproline residues. The ferric chloride-orcinol assay (Bial's test), which is specific for pento...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-006-6217-2
更新日期:2006-11-01 00:00:00
abstract::Two Arabidopsis xylosyltransferases, designated RGXT1 and RGXT2, were recently expressed in Baculovirus transfected insect cells and by use of the free sugar assay shown to catalyse transfer of D-xylose from UDP-alpha-D-xylose to L-fucose and derivatives hereof. We have now examined expression of RGXT1 and RGXT2 in Pi...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-009-9242-0
更新日期:2009-12-01 00:00:00
abstract::The carbohydrate determinants, sialyl Lewis A and sialyl Lewis X, which are frequently expressed on human cancer cells, serve as ligands for a cell adhesion molecule of the selectin family, E-selectin, which is expressed on vascular endothelial cells. These carbohydrate determinants are involved in the adhesion of can...
journal_title:Glycoconjugate journal
pub_type: 杂志文章,评审
doi:10.1023/a:1018532409041
更新日期:1997-08-01 00:00:00
abstract::Influenza virus neuraminidase inhibitors (NAIs), currently used as anti-influenza drugs, can lead to the appearance of drug-resistant variants. Resistance to NAIs appears due to mutations in the active site of the neuraminidase (NA) molecule that decrease the NA enzymatic activity and sometimes in the hemagglutinin (H...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/s10719-010-9280-7
更新日期:2010-04-01 00:00:00
abstract::The novel glycosphingolipid, SEGLx (Galbeta1-4(Fucalpha1-3)Glcbeta1-3GalbetaCer), which was identified by us (Kawakami Y, et al. (1993) J Biochem 114: 677-83), shows a characteristic spectrum on 1H-NMR analysis, in which the anomeric proton resonances of a reducing end galactose and a glucose are split. To elucidate t...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/a:1006908020370
更新日期:1998-02-01 00:00:00
abstract::In this report we present the enzymatic characterization of CMP-NeuAc:Gal beta 1-4GlcNAc-R alpha(2-3)-sialyltransferase from human placenta using placenta membranes as an enzyme preparation. This sialyltransferase is highly sensitive to detergents and prefers type 2 chain (Gal beta 1-4GlcNAc) over type 1 chain (Gal be...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731193
更新日期:1993-02-01 00:00:00
abstract::The TF, Tn, and SiaTn glycotopes are frequently expressed in cancer-associated mucins. Antibodies to these glycotopes were found in human serum. A set of polyacrylamide (PAA)--based glycoconjugates was applied to the direct and competitive enzyme-linked immunosorbent assays (ELISA) to characterize the specificity of s...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1023/B:GLYC.0000018582.83813.04
更新日期:2004-01-01 00:00:00
abstract::Comparing the properties of 'young' and senescent ('aged') O+ erythrocytes isolated by applying ultracentrifugation in a self-forming Percoll gradient, we demonstrate that the sialic acids of membrane glycoconjugates control the life span of erythrocytes and that the desialylation of glycans is responsible for the cle...
journal_title:Glycoconjugate journal
pub_type: 杂志文章
doi:10.1007/BF00731328
更新日期:1995-06-01 00:00:00