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1

Otrzymanie wysoko oczyszczonych substancji grupowych A z krwinek czerwonych ludzkich

100%
Koscielak J.
Postępy Biochemii
|
1962
|
tom 08
|
nr 4
2

Diseases of aberrant glycosylation

100%
Koscielak J.
Acta Biochimica Polonica
|
1995
|
tom 42
|
nr 1
Recently a defective glycosylation of glycoconjugates has been implicated in the pathogenesis of a number of heritable or acquired diseases of humans. Herein I discuss them under the name of diseases of aberrant glycosylation. These are: conge­nital dyserythropoietic anemia type II, carbohydrate-deficient glycoprotein syndrome, I-cell disease, galactosemia in subjects on galactose-free diet, variants of leukocyte adhesion deficiency, and of Ehlers-Danlos syndrome, paroxysmal nocturnal hemo­globinuria, and Tn syndrome. Regarding the present views on the function of glyco­conjugates it is probably significant that in most instances defective or missing glycoproteins (or proteoglycans,) but not glycosphingolipids, are probably involved in the pathogenesis of these diseases.
3

Carbohydrate-deficient glycoprotein syndromes

100%
Koscielak J.
Acta Biochimica Polonica
|
1999
|
tom 46
|
nr 3
Carbohydrate-deficient glycoprotein syndromes are rare, multisystemic diseases, typically with major nervous system impairment, that are caused by hypo-and unglycosylation of N-linked glycoproteins. Hence, a biochemical evidence of this abnormality, like hypoglycosylation of serum transferrin is essential for diagnosis. Clinically and biochemically, six types of the disease have been delineated. Three of them are caused by deficiencies of the enzymes that are required for a proper glycosylation of lipid - (dolichol) linked oligosaccharide (phosphomannomutase or phosphomannose isomerase or alpha-glycosyltransferase), and one results from a deficiency of Golgi resident N-acetylglucosaminyltransferase II. In addition one variant of the disease has been reported as due to a defective biosynthesis of dolichol iself. The diseases are heritable but genetics has been established for only two types. Therapy, based on administration of mannose to patients is currently under investigation. It benefits patients with deficiency of phosphomannose isomerase. Taking into account the complexity of N-linked glycosylation of proteins more of the disease variants is expected to be found.
4
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Substancja A z krwinek czerwonych

63%
Koscielak J., Zakrzewski K.
Acta Physiologica Polonica
|
1957
|
tom 08
|
nr 3-3a
5

Wpływ oczyszczania na aktywność sybstancji grupowej A z erytrocytów ludzkich

51%
Koscielak J., Grochowska E., Zakrzewski K.
Postępy Biochemii
|
1962
|
tom 08
|
nr 4
6
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Content available

Oznaczanie glukozy we krwi metodą "Fenol-kwas siarkowy"

51%
Skalska-Rakowska J., Grochowska E., Czartoryska B., Koscielak J.
Diagnostyka Laboratoryjna
|
1968
|
tom 04
|
nr 1
7

Oznaczanie objętości krwi krążącej za pomocą dekstranu

51%
Czartoryska B., Koscielak J., Kozlowski S., Panasewicz J.
Acta Physiologica Polonica
|
1965
|
tom 16
|
nr 5
8

In comparison to progenitor platelets, microparticles are deficient in GpIb, GpIb-derived carbohydrates, glycerophospholipids, glycosphingolipids, and ceramides

51%
Zdebska E., Wozniak J., Dzieciatkowska A., Koscielak J.
Acta Biochimica Polonica
|
1998
|
tom 45
|
nr 2
Activated blood platelets shed microparticles with procoagulant activity that probably participate in normal hemostasis. We have isolated spontaneously formed microparticles from human blood and analysed them for ultrastructure, antigenic profile, and biochemical composition. In transmission electron microscopy microparticles appeared as regular vesicles with a mean diameter of 300 nm (50-600 nm). In flow cytometry almost all microparticles reacted with fluorescein isothiocyanate (FITC) labeled antibody to platelet glycoprotein complex IIb-IIIa (GpIIb-IIIa) and with FITC-annexin V but only 40-50% of microparticles reacted with FITC-antibody to platelet glycoprotein Ib (GpIb). The latter result was confirmed by double labeling of microparticles with FITC-antibody to GpIIb-IIIa and phycoerythrin (PE) labeled antibody to GpIb. Large microparticles reacted better with anti-GpIb than the small ones. A decreased level of GpIb was also demonstrated by SDS/polyacrylamide gel electrophoresis of microparticles. Compositional studies indicated, that in terms of cholesterol and protein contents, microparticles resembled platelets rather than platelet membranes as previously thought. They are, however, deficient in certain components. Thus, in comparison to platelets, microparticles had reduced contents of sialic acid (by 56.4%), galactosamine (by 48.2%), glucosamine (by 22.4%), galactose by (11.8%) and fucose (by 21.6%). Mannose content was increased by 11.8%. Total phospholipids in microplatelets were lower by 17.8%. Glycerophospholipids only were affected with phosphatidylserine being decreased as much as by 43.2%. Neutral glycosphingolipids, gangliosides and ceramides in microparticles were reduced by half. We conclude that the biochemical composition of microparticles probably reflects previous activation of progenitor platelets
9

The levels of glycosphingolipids, ceramides, sialic acid and glycogen are changed in plasma membranes of rat platelets harvested during recovery from immune-mediated thrombocytopenia

51%
Zdebska E., Soszynska B., Dobrowolski Z., Koscielak J.
Acta Biochimica Polonica
|
1996
|
tom 43
|
nr 3
Plasma membranes of rat platelets produced at normal platelet counts and during early recovery from immune-mediated thrombocytopenia were examined for the contents of carbohydrates, lipids and glycosphingolipids. Glucosylceramide, two monosialo-gangliosides and one disialo-ganglioside were found to be the major glycosphingolipids of platelets. During thrombocytopenia the contents of these glycosphingolipids as well as of ceramides were several fold elevated. Among carbohydrate constituents of platelets and platelet plasma membranes, glycogen content was increased and that of sialic acid decreased. These results are discussed in the light of literature data on relevant biochemical characteristics of megakaryocytes at different stages of maturation and on thrombopoiesis during acute experimental thrombocytopenia.
10

The activity of alpha 1,6-fucosyltransferase during human megakaryocytic differentiation

51%
Bany-Laszewicz U., Kaminska J., Klimczak-Jajor E., Koscielak J.
Cellular and Molecular Biology Letters
|
2004
|
tom 09
|
nr 1
α1,6-Fucosyltransferase (6FucT, E.C. 2.4.1.68) is one of the enzymes involved in the synthesis of N-linked glycans of the GpIIb/IIIa complex (CD41a) which is present on megakaryocytes (MKs) and platelets. In this study, we examined 6FucT activity in ex vivo cultures of immunoselected cord blood CD34+ cells grown in a medium promoting megakaryocytopoiesis. Our results show that the activity of 6FucT increased ahead of, and thereafter concomitantly with, cells expressing the CD41a antigen. When the CD41a+ subpopulation of cells was immunoselected (using anti-CD61 i.e. anti-GpIIIa antibodies), its 6FucT activity increased proportionally to the yield of CD61+ cells. Taking into account the heavy load of 6FucT in platelets and megakaryocytes, we regard this enzyme as a candidate for the earliest marker of MK-commitment in cultured hematopoietic stem cells. Such a marker should allow an earlier detection and earlier transplantation of patients’ own, ex vivo expanded, Mk progenitors.
11

Glycophorin A in two patients with congenital dyserythropoietic anemia type I and type II is partly unglycosylated

51%
Zdebska E., Adamczyk-Poplawska M., Koscielak J.
Acta Biochimica Polonica
|
2000
|
tom 47
|
nr 3
Glycophorins A from erythrocyte membranes of two patients with congenital dyserythropoietic anemia type I and type II (CDA type I and II) were analyzed for carbohydrate molar composition employing a modification of the recently published method that allowed simultaneous determination of carbohydrates and protein in electrophoretic bands of glycoproteins separated by sodium dodecyl sulfate- polyacrylamide gel electrophoresis (Zdebska & Koscielak, 1999, Anal. Biochem., 275, 171-179). The modification involved a preliminary extraction of erythrocyte membranes with aqueous phenol, subsequent electrophoresis and analysis of the extracted glycophorins rather than electrophoresis and analysis of the glycophorin from intact erythrocyte membranes. The results showed a large deficit of N-acetylgalactosamine, galactose, and sialic acid residues in glycophorin A from patients with CDA type I and type II amounting to about 45% and 55 %, respectively. The results strongly suggest that glycophorin A in these patients is partly unglycosylated with respect to O-linked glycans. In addition, glycophorin A from erythrocytes of a patient with CDA II but not CDA I exhibited a significant deficit of mannose and N-acetylglucosamine suggesting that its N-glycosylation site was also partly unglycosylated.
12

Paul-Bunnell antigen and a possible mechanism of formation of heterophile antibodies in patients with infectious mononucleosis

51%
Golaszewska E., Kurowska E., Duk M., Koscielak J.
Acta Biochimica Polonica
|
2003
|
tom 50
|
nr 4
Sera of patients with infectious mononucleosis contain heterophile anti-Paul- Bunnell (PB) antibodies to erythrocytes of numerous mammalian species. Evidence is presented that the corresponding antigen of bovine erythrocytes is not, as previ­ously described, a single molecule, but a series of glycoproteins with glycans termi­nated with .-glycolylneuraminic acid (Neu5Gc). The latter compound should be an important part of the PB epitope because, in agreement with the results of others, we found that desialylation of the PB antigen abolishes almost completely its activity. We examined three different preparations of GM3 ganglioside for their capacity to bind anti-PB and found that only GM3 from horse erythrocytes containing Neu5Gc exhibited a low although ELISA measurable PB activity. The other two GM3 prepa­rations, from bovine milk and dog erythrocytes, containing .-acetylneuraminic acid (Neu5Ac) bound little if any anti-PB antibodies. This finding confirms a previous re­port that human erythrocyte Neu5Ac containing sialoglycoprotein with similar O-linked glycans as the PB-antigen of bovine erythrocytes exhibits only very low PB activity (Patarca & Fletcher, 1995, Crit Rev Oncogen., 6: 305). In conclusion, we present a hypothesis that anti-PB antibodies in patients with infectious mononucleosis are formed against infection-induced cell membrane glycoconjugates containing highly immunogenic Neu5Gc.
13

Monitoring of chimerism in patients after allogencic bone marrow transplation employing AmpF/STR SGM Plus kit

45%
Sawecka J., Skulimowska J., Halaburda K., Marianska B., Koscielak J.
Acta Biochimica Polonica
|
2006
|
tom 53
|
nr Supplement 1
14

Ocena dojrzewania komorek progenitorowych megakariocytow w hodowli in vitro komorek macierzystych hematoporezy

45%
Bany-Laszewicz U., Klimczak-Jajor E., Palynyczko G., Koscielak J., Kaminska J.
Postępy Biologii Komórki. Suplement
|
2003
|
tom 21
61-71
15

Prevalence of the intron 22 inversion of the factor VIII gene and inhibitor development in Polish patients with severe hemophilia A

45%
Sawecka J., Skulimowska J., Windyga J., Lopaciuk S., Koscielak J.
Archivum Immunologiae et Therapiae Experimentalis
|
2005
|
tom 53
|
nr 4
16

Activity of platelet alpha-6-fucosyltransferase is inversely related to blood platelet concentration

45%
Koscielak J., Antoniewicz-Papis J., Zdebska E., Maj S., Leszko B.
Acta Biochimica Polonica
|
1995
|
tom 42
|
nr 1
The activity of serum a-6-fucosyl transferase, a platelet derived enzyme, determined in sera of 22 normal individuals and 86 patients with various disorders was positively correlated with platelet counts. When the enzyme activity in 1 jil serum was calculated per 1000 of platelets in blood (coefficient F/P) an inverse correlation became evident in that F/P was proportionally the higher the lower was platelet count in blood. The F/P values were in a good agreement with the results of direct assays of enzyme activities in isolated platelets. Neither granulocytes, lymphocytes nor red cells signi­ficantly contributed to serum enzyme activity though granulocytes enhanced the thrombin-induced enzyme release from platelets. In platelets separated by centri- fugation in density gradients the enzyme was shown to be present in platelets of intermediate and high density but missing from the light ones. It is suggested that a-6-fucosyltransferase of platelets may be a marker of the ploidy level of mega­karyocytes.
17

Quantitative analysis of LacCer-CDw17 in human myelogenous leukaemic cells

45%
Spychalska J., Smolenska-Sym G., Zdebska E., Wozniak J., Koscielak J.
Cellular and Molecular Biology Letters
|
2003
|
tom 08
|
nr 4
LacCer/CDw17 is the most abundant GSL in neutrophils. The cell-surface and intracellular presence of LacCer was determined quantitatively using anti-CDw17 mAbs in a flow cytometry assay. The quantified alterations in the level of CDw17 antigen expression are consistent with alterations in LacCer content, determined chemically. Our results show that CDw17 antigen expression defines successive stages in the maturation of the myeloid cell. The assessment of cell-surface and intracellular CDw17 expression may be useful in evaluating neutrophil physiological status.
18

Src kinases inhibitor, SU6656, induces differentiation and maturation of megakaryocyte progenitor cells

39%
Kaminska J., Bany-Laszewicz U., Klimczak-Jajor E., Skierski J., Zdebska E., Koscielak J.
Acta Biochimica Polonica
|
2007
|
tom 54
|
nr Supplement 4
19

Contents of total and protein-bound carbohydrates are low in leukemic leucocytes from patients with acute myelogenous leukemia

33%
Smolenska-Sym G., Zdebska E., Golaszewska E., Wozniak J., Durzynski T., Maj S., Mokras U., Koscielak J.
Acta Biochimica Polonica
|
1998
|
tom 45
|
nr 2
Leukemic leukocytes from 12 patients with acute myelogenous leukemia (AML) and two patients with chronic myelogenous leukemia (CML) were isolated by centrifugations in Percoll gradients, and examined for total carbohydrates. In leukemic leukocytes from 10 of these patients ceramide-bound carbohydrates were also determined. Protein-bound carbohydrates were calculated by subtraction of ceramide-bound carbohydrates from total carbohydrates. In all samples analysed the contents of total and protein-bound carbohydrates were much lower in leukemic leukocytes than in normal neutrophils, irrespective whether the results were expressed relative to protein, DNA, cell number or dry mass. For immature leukemic cells of MO-M1 phenotype differences up to 10-fold were observed. Contents of ceramide-bound carbohydrates, i.e. those of neutral and acidic glycosphingolipids (GSLs) were also low in leukemic cells. However, when GSL carbohydrates were calculated as percentage of total carbohydrates, GSLs in leukemic leukocytes were elevated in half of the AML patients but depressed in the other half. The results are discussed in the light of the hypothesis on GSL function by one of us (Kościelak J., 1986, Glycoconjugate J. 3, 95-108). According to one element of the hypothesis, during cell differentiation newly synthesized glycoproteins (GPs) that perform specific functions are added to house-keeping GPs that are present in plasma membranes of all types of cells. Thus, during differentiation, the GP content of the cell membrane should increase and that of the so called "membrane packing" glycosphingolipids should decrease.
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