Isolation and characterization of poly-N-acetyllactosaminylceramides accumulated in the erythrocytes of congenital dyserythropoietic anemia type II patients.
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| Abstract |
Congenital dyserythropoietic anemia type II or hereditary erythroblastic polynuclearity with positive acidified serum test (HEMPAS) is a rare genetic disease inherited by a recessive mode. Previous studies on HEMPAS erythrocytes have shown that Band 3 and Band 4.5 glycoproteins were not glycosylated by lactosaminoglycans, while polylactosaminyl carbohydrates are accumulated as glycolipids (P. Scartezzini et al., Br J. Haematol., 51 (1982) 569; M.N. Fukuda et al., Br. J. Haematol., 56 (1984)55). Presently, we have isolated polylactosaminyl lipids from HEMPAS blood cells and analyzed their structures by fast atom bombardment-mass spectrometry (FAB-MS), methylation analysis, endo-beta-galactosidase digestion. The results indicate that polylactosaminyl lipids accumulated in HEMPAS erythrocytes are a species of poly-N-acetyllactosaminylceramides which are also present in normal erythrocytes, but at 7 approximately 9 times lower level. Isolated polylactosaminylceramides exhibit I-, i-, H- and Lex antigenic activities which suggest that the polylactosaminylceramides are derived from both erythrocytes and granulocytes.
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| Authors | M N Fukuda, B Bothner, P Scartezzini, A Dell |
| Journal | Chemistry and physics of lipids (Chem Phys Lipids) Vol. 42 Issue 1-3 Pg. 185-97 (Dec 15 1986) ISSN: 0009-3084 [Print] Ireland |
| PMID | 3829205 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.) |
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