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001-es BibID:	BIBFORM034624
Első szerző:	Balogh István (molekuláris biológus, genetikus)
Cím:	Val34Leu polymorphism of plasma factor XIII : biochemistry and epidemiology in familial thrombophilia / István Balogh, Gabriella Szőke, Levente Kárpáti, Ulla Wartiovaara, Éva Katona, István Komáromi, Gizella Haramura, György Pfliegler, Hanna Mikkola, László Muszbek
Dátum:	2000
ISSN:	0006-4971
Megjegyzések:	Val34Leu polymorphism of the A subunit of coagulation factor XIII (FXIII-A) is located in the activation peptide (AP) just 3 amino acids away from the thrombin cleavage site. This mutation has been associated with a protective effect against occlusive arterial diseases and venous thrombosis; however, its biochemical consequences have not been explored. In the current study it was demonstrated that the intracellular stability and the plasma concentration of FXIII of different Val34Leu genotypes are identical, which suggests that there is no difference in the rate of synthesis and externalization of wild-type and mutant FXIII-A. In contrast, the release of AP by thrombin from the Leu34 allele proceeded significantly faster than from its wild-type Val34 counterpart. By molecular modeling larger interaction energy was calculated between the Leu34 variant and the respective domains of thrombin than between the Val34 variant and thrombin. In agreement with these findings, the activation of mutant plasma FXIII by thrombin was faster and required less thrombin than that of the wild-type variant. Full thrombin activation of purified plasma FXIII of different genotypes, however, resulted in identical specific transglutaminase activities. Similarly, the mean specific FXIII activity in the plasma was the same in the groups with wild-type, heterozygous, and homozygous variants. Faster activation of the Leu34 allele hardly could be associated with its presumed protective effect against venous thrombosis. No such protective effect was observed in a large group of patients with familial thrombophilia.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Blood. - 96 : 7 (2000), p. 2479-2486. -
További szerzők:	Szőke Gabriella Kárpáti Levente (1968-) (okleveles vegyész) Wartiovaara, Ulla Katona Éva (1961-) (klinikai biokémikus) Komáromi István (1957-) (vegyész, molekuláris biológus, biokémikus) Haramura Gizella (1957-) (vezető analitikus) Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Mikkola, Hanna Muszbek László (1942-) (haematológus, kutató orvos)
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001-es BibID:	BIBFORM034614
035-os BibID:	PMID:1799664
Első szerző:	Boda Zoltán (belgyógyász, haematologus, klinikai onkológus)
Cím:	Treatment of the severe bleeding episode in type III von Willebrand's disease by simultaneous administration of cryoprecipitate and platelet concentrate : case report / Z. Boda, G. Pfliegler, J. Hársfalvi, K. Rak
Dátum:	1991
ISSN:	0957-5235
Megjegyzések:	A severe, life-threatening bleeding episode in a 24-year-old woman suffering from type III von Willebrand's disease was treated by large doses of cryoprecipitate with unsatisfactory results. Bleeding ceased and the bleeding time normalized only after concomitant administration of platelet concentrates. In the treatment of von Willebrand's disease patients possessing platelets with absent or insufficient von Willebrand factor activity the administration of plasma concentrates together with platelets appears to be justified.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok esettanulmány vWF type III platelet vWF activity cryoprecipitate platelet substitution combined treatment egyetemen (Magyarországon) készült közlemény
Megjelenés:	Blood Coagulation & Fibrinolysis. - 2 : 6 (1991), p. 775-777. -
További szerzők:	Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Hársfalvi Jolán (1949-) (klinikai biokémikus) Rák Kálmán (1929-2005) (belgyógyász, klinikai hematológus, véralvadás kutató)
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001-es BibID:	BIBFORM034608
035-os BibID:	PMID:2133212
Első szerző:	Hársfalvi Jolán (klinikai biokémikus)
Cím:	The use of polybrene for heparin neutralization in protein C activity assay / J. Harsfalvi, G. Pfliegler, M. Udvardy, Z. Boda, I. Tornai, K. Rak
Dátum:	1990
ISSN:	0957-5235
Megjegyzések:	The protein C activity assay of Francis and Patch (Thromb Res 1983; 32: 605-613) is based on the prolongation of the activated partial thromboplastin time in the presence of activated protein C isolated from the test samples. The assay was modified and standardized by Rapaport et al. (Am J Clin Pathol 1987; 87: 491-497), but could still only be used in patients on heparin therapy after chromatographic removal of the heparin. In this study we attempted to eliminate the heparin separation step without losing the advantages of the modified (Rapaport) method. Heparin was added to the isolated protein C to obtain a rapid and complete antithrombin effect after the thrombin activation step and polybrene was subsequently used to neutralize the excess heparin. Using this modified assay protein C activity ranged from 67 to 133% in the normal population, and from 9 to 25% in coumarin-treated patients. Precision of the modified method was acceptable in both normal and pathological PC ranges: within- and between-batch variations were 5.6 and 3.6%, and 8 and 14%, respectively. The assay correlated well (r = 0.84) with the ELISA technique in both healthy donors and non-coumarin-treated patients.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban Protein C assay protein C deficiency thrombosis anticoagulant therapy egyetemen (Magyarországon) készült közlemény
Megjelenés:	Blood Coagulation & Fibrinolysis. - 1 : 4-5 (1990), p. 357-361. -
További szerzők:	Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Udvardy Miklós (1947-) (belgyógyász, haematológus) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Tornai István (1954-) (belgyógyász, gasztroenterológus) Rák Kálmán (1929-2005) (belgyógyász, klinikai hematológus, véralvadás kutató)
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001-es BibID:	BIBFORM034573
035-os BibID:	PMID:3028538 WOS:A1987G302900025
Első szerző:	Kienast, Joachim
Cím:	Sodium fluoride mimics effects of both agonists and antagonists on intact human platelets by simultaneous modulation of phospholipase C and adenylate cyclase activity / Joachim Kienast, Jef Arnout, György Pfliegler, Hans Deckmyn, Bernard Hoet, Jos Vermylen
Dátum:	1987
ISSN:	0006-4971
Megjegyzések:	Using intact human platelets, we studied the effect of sodium fluoride (NaF) on platelet aggregation and release reaction and correlated the functional changes to intracellular events specific for either agonist-induced or antagonist-induced platelet responses. At lower concentrations, with a peak activity between 30 and 40 mmol/L, NaF induced aggregation and release of adenosine 5'-triphosphate (ATP) that was associated with increased formation of inositol phosphates, a rise in cytosolic free Ca2+, and phosphorylation of 20-kd and 40-kd proteins. At NaF concentrations greater than 40 mmol/L, aggregation and ATP release decreased dose-dependently in parallel with a decrease in Ca2+ mobilization, whereas neither inositol phosphate formation nor 40-kd protein phosphorylation was reduced. At these concentrations, NaF caused a dose-dependent transient rise in platelet cyclic adenosine 3',5'-monophosphate (cAMP) levels that was sufficient to account for the observed reduction in Ca2+ mobilization, aggregation, and ATP release. Stimulated cAMP levels started declining rapidly within 30 seconds of addition of NaF, however. Similarly, prostacyclin (PGI2)-induced cAMP accumulation was temporarily enhanced but subsequently suppressed by NaF, suggesting either stimulation of a cAMP phosphodiesterase or delayed inhibition of adenylate cyclase. Evidence for the latter was provided by the finding that NaF pretreatment of platelets resulted in partial inhibition of PGI2-stimulated cAMP formation in the presence of the cAMP phosphodiesterase inhibitor 3-isobutyl-1-methyl-xanthine (MIX). We conclude that NaF exerts a dual (stimulatory and inhibitory) effect on adenylate cyclase in intact platelets that is accompanied by simultaneous activation of a phosphoinositide-specific phospholipase C; in addition, a cAMP phosphodiesterase may be activated.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban külföldön készült közlemény
Megjelenés:	Blood. - 69 : 3 (1987), p. 859-866. -
További szerzők:	Arnout, Jef Pfliegler György (1949-) (belgyógyász, hematológus, labor szakorvos) Deckmyn, Hans Hoet, Bernard Vermylen, Jozef
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