Blood group B glycosphingolipids in α-galactosidase deficiency (Fabry disease): influence of secretor status
Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal α-galactosidase EC 3.2.1.22) has been studied using highly sensitive and specific TLC-immunostaining analysis of urinary sediments and tonsillar tissues of blood group B patients and heal...
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Published in | Biochimica et biophysica acta Vol. 1345; no. 2; pp. 180 - 187 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
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Elsevier B.V
01.04.1997
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Abstract | Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal
α-galactosidase EC 3.2.1.22) has been studied using highly sensitive and specific TLC-immunostaining analysis of urinary sediments and tonsillar tissues of blood group B patients and healthy controls, secretors and nonsecretors. The B glycolipid antigens with hexasaccharide chains were consistently found increased (25- to 100-fold) in the urinary sediments of three Fabry patients, blood group B or AB secretors. Conversely, they were absent in the urinary sediment of one blood group B nonsecretor patient. In normal secretors, B glycosphingolipids were present only in traces. Moreover, significant increase in B glycolipid antigens (8-fold) was found in the tonsillar tissue of a Fabry patient blood group B secretor. We conclude that the secretor status is responsible for increased concentration of blood group B glycosphingolipids in both urinary cells and tonsils in
α-galactosidase deficiency. The quantity of stored B-immunoactive glycosphingolipids, however, is much lower than that of the mainly accumulated glycosphingolipid Gb
3Cer. The results clearly indicate that active or silent
Se gene, which controls synthesis of B-antigen precursors, is responsible for notable difference in B-glycosphingolipids expression in Fabry patients – secretors and nonsecretors. Whether this novel aspect may be of prognostic significance, remains to be established. © 1997 Elsevier Science B.V. All rights reserved. |
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AbstractList | Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal alpha-galactosidase EC 3.2.1.22) has been studied using highly sensitive and specific TLC-immunostaining analysis of urinary sediments and tonsillar tissues of blood group B patients and healthy controls, secretors and nonsecretors. The B glycolipid antigens with hexasaccharide chains were consistently found increased (25- to 100-fold) in the urinary sediments of three Fabry patients, blood group B or AB secretors. Conversely, they were absent in the urinary sediment of one blood group B nonsecretor patient. In normal secretors, B glycosphingolipids were present only in traces. Moreover, significant increase in B glycolipid antigens (8-fold) was found in the tonsillar tissue of a Fabry patient blood group B secretor. We conclude that the secretor status is responsible for increased concentration of blood group B glycosphingolipids in both urinary cells and tonsils in alpha-galactosidase deficiency. The quantity of stored B-immunoactive glycosphingolipids, however, is much lower than that of the mainly accumulated glycosphingolipid Gb(3)Cer. The results clearly indicate that active or silent Se gene, which controls synthesis of B-antigen precursors, is responsible for notable difference in B-glycosphingolipids expression in Fabry patients - secretors and nonsecretors. Whether this novel aspect may be of prognostic significance, remains to be established. Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal α-galactosidase EC 3.2.1.22) has been studied using highly sensitive and specific TLC-immunostaining analysis of urinary sediments and tonsillar tissues of blood group B patients and healthy controls, secretors and nonsecretors. The B glycolipid antigens with hexasaccharide chains were consistently found increased (25- to 100-fold) in the urinary sediments of three Fabry patients, blood group B or AB secretors. Conversely, they were absent in the urinary sediment of one blood group B nonsecretor patient. In normal secretors, B glycosphingolipids were present only in traces. Moreover, significant increase in B glycolipid antigens (8-fold) was found in the tonsillar tissue of a Fabry patient blood group B secretor. We conclude that the secretor status is responsible for increased concentration of blood group B glycosphingolipids in both urinary cells and tonsils in α-galactosidase deficiency. The quantity of stored B-immunoactive glycosphingolipids, however, is much lower than that of the mainly accumulated glycosphingolipid Gb 3Cer. The results clearly indicate that active or silent Se gene, which controls synthesis of B-antigen precursors, is responsible for notable difference in B-glycosphingolipids expression in Fabry patients – secretors and nonsecretors. Whether this novel aspect may be of prognostic significance, remains to be established. © 1997 Elsevier Science B.V. All rights reserved. |
Author | Poupětová, Helena Ledvinová, Jana Elleder, Milan Hanáčková, Alžběta Pı́sačka, Martin |
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BackLink | https://www.ncbi.nlm.nih.gov/pubmed/9106497$$D View this record in MEDLINE/PubMed |
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Cites_doi | 10.1159/000460912 10.1016/S0950-3536(06)80038-7 10.1016/S0022-5347(17)43038-2 10.1056/NEJM197104082841401 10.1016/0304-4157(94)90017-5 10.1177/014107688908200918 10.1016/0076-6879(94)30030-5 10.3109/01913129009076133 10.1016/0304-3886(92)90061-W 10.1007/BF01330831 10.1016/0005-2760(80)90227-1 10.1159/000461485 10.1126/science.167.3922.1268 10.1016/0925-4439(92)90024-H 10.1097/00007890-198607000-00022 10.1007/978-1-4757-9537-0_2 10.1016/0076-6879(82)83012-7 10.1016/S0021-9258(19)44007-6 10.1016/S0022-2275(20)43219-5 10.1016/0076-6879(78)50022-0 10.1016/S0021-9258(17)45285-9 10.1021/bi00335a028 10.1093/ajcp/82.1.24 |
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Keywords | Urinary sediment Tonsil B gene, blood group B gene HPTLC, high-performance thin-layer chromatography Gg 4Cer (or GA1), asialoGM1 ganglioside mAbs, monoclonal antibodies Fabry disease Glycosphingolipid antigen Ga 2Cer, galabiosylceramide LacCer, lactosylceramide LSIMS, liquid-secondary ion mass spectrometry Gb 4Cer, globoside Gb 3Cer, globotriaosylceramide α-Galactosidase deficiency HGC, hexaglycosylceramide Se gene, secretor gene GSL, glycosphingolipids Blood group B secretor GlcCer, glucosylceramide B-GSL, glycosphingolipids with blood group B determinant B-6 (or A-6), shortened designation for hexaglycosylceramide with blood group B (or A) determinant based on both types of oligosaccharide chain: type 1 (B-6-1, IV 2- α-fucosyl-IV 3- α-galactosyl-lactotetraglycosylceramide): Gal α1→3Gal(2←1Fuc) β1→3GlcNAc β1→3Gal β1→4Glc β1→1́Cer; type 2 (B-6-2, IV 2- α-fucosyl-IV 3- α-galactosyl-neolactotetraglycosylceramide): Gal α1→3Gal(2←1Fuc) β1→4GlcNAc β1→3Gal β1→4Glc β1→1́Cer (terminal monosaccharide in A determinant is GalNAc α instead of Gal α) Neutral glycosphingolipids are abbreviated according to the recommendation of the IUPAC-IUB Commission on Biochemical Nomenclature Eur. J. Biochem. 79, 11–21 |
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Snippet | Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal
α-galactosidase EC 3.2.1.22) has been studied... Defect in degradation of blood group B-immunoactive glycosphingolipids in Fabry disease (deficiency of lysosomal alpha-galactosidase EC 3.2.1.22) has been... |
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SubjectTerms | ABO Blood-Group System - blood ABO Blood-Group System - urine Adult Animals Antibodies, Monoclonal - immunology Blood group B secretor Chromatography, Thin Layer Fabry disease Fabry Disease - blood Fabry Disease - urine Glycosphingolipid antigen Glycosphingolipids - analysis Glycosphingolipids - chemistry Glycosphingolipids - immunology Glycosphingolipids - metabolism Humans Kidney Transplantation Mice Palatine Tonsil - chemistry Reference Values Time Factors Tonsil Urinary sediment α-Galactosidase deficiency |
Title | Blood group B glycosphingolipids in α-galactosidase deficiency (Fabry disease): influence of secretor status |
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