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001-es BibID:	BIBFORM007182
Első szerző:	Ajzner Éva (laboratóriumi szakorvos)
Cím:	Severe bleeding complications caused by an autoantibody against the B subunit of plasma factor XIII : a novel form of acquired factor XIII deficiency / Éva Ajzner, Ágota Schlammadinger, Adrienne Kerényi, Zsuzsanna Bereczky, Éva Katona, Gizella Haramura, Zoltán Boda, László Muszbek
Dátum:	2009
Megjegyzések:	Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of FXIII (FXIII-B) that caused life-threatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A(2)B(2) complex, and individual FXIII subunits were undetectable in the plasma, whereas platelet FXIII activity and antigen were normal. Neither FXIII activation nor its activity was inhibited by the antibody, which bound to structural epitope(s) on both free and complexed FXIII-B. The autoantibody highly accelerated the elimination of FXIII from the circulation. FXIII supplementation combined with immunosuppressive therapy, plasmapheresis, immunoglobulin, and anti-CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Blood. - 113 : 3 (2009), p. 723-725. -
További szerzők:	Schlammadinger Ágota (1971-) (belgyógyász, haematológus) Kerényi Adrienne (1970-) (laboratóriumi szakorvos) Bereczky Zsuzsanna (1974-) (orvosi laboratóriumi diagnosztika szakorvos) Katona Éva (1961-) (klinikai biokémikus) Haramura Gizella (1957-) (vezető analitikus) Boda Zoltán (1947-) (belgyógyász, haematologus, klinikai onkológus) Muszbek László (1942-) (haematológus, kutató orvos)
Internet cím:	elektronikus változat DOI elektronikus változat
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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)
Internet cím:	Intézményi repozitóriumban (DEA) tárolt változat Szerző által megadott URL
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001-es BibID:	BIBFORM006762
Első szerző:	Inbal, Aida
Cím:	Platelets but not monocytes contribute to the plasma levels of factor XIII subunit A in patients undergoing autologous peripheral blood stem cell transplantation / Inbal, A., Muszbek, L., Lubetsky, A., Katona, E., Levi, I., Karpati, L., Nagler, A.
Dátum:	2004
ISSN:	0957-5235
Megjegyzések:	Factor XIII subunit A (FXIII A) is synthesized by megakaryocytes, and monocytes/macrophages. In addition, the liver has been reported as an extrahaematopoietic source of FXIII A. At present, the extent of contribution of either haematopoietic or extrahaematopoietic sources to the plasma FXIII A level is unknown. We studied the effect of bone marrow aplasia due to high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (ASCT) on plasma FXIII A activity and concentration in 20 patients with haematological or solid tumour malignancies. A multiple linear regression model was used to assess the effect of gender, age, malignancy and treatment types, platelet and monocyte counts, abnormal liver function tests, prothrombin time, and number of platelet transfusions on FXIII activity measured in plasma before and following ASCT. Significant correlation between platelet counts and FXIII A activity in plasma was observed (r = 0.51, P = 0.0001), which remained after the adjustment for the aforementioned parameters (multiple R = 0.52, P = 0.0001). In contrast, no significant correlation between FXIII A levels and monocyte counts was observed (r = 0.19), and this lack of correlation persisted after the adjustment. These results suggest that in the ASCT model, following myeloablation, platelets but not monocytes are the haematopoietic cells that contribute significantly to plasma FXIII A levels. In addition, extra-haematopoietic sources of FXIII synthesis may also contribute to FXIII levels in plasma.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban Blood Platelets Factor XIII Hematologic Neoplasms Humans Leukocyte Count Monocytes Peripheral Blood Stem Cell Transplantation Platelet Count Regression Analysis Time Factors Transplantation, Autologous
Megjelenés:	Blood Coagulation and Fibrinolysis. - 15 : 3 (2004), p. 249-253. -
További szerzők:	Muszbek László (1942-) (haematológus, kutató orvos) Lubetsky, Aharon Katona Éva (1961-) (klinikai biokémikus) Levi, Itai Kárpáti Levente (1968-) (okleveles vegyész) Nagler, Arnon
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001-es BibID:	BIBFORM049371
035-os BibID:	PMID:24408323
Első szerző:	Katona Éva (klinikai biokémikus)
Cím:	Interaction of factor XIII subunits / Éva Katona, Krisztina Pénzes, Andrea Csapó, Ferenc Fazakas, Miklós L. Udvardy, Zsuzsa Bagoly, Zsuzsanna Z. Orosz, László Muszbek
Dátum:	2014
ISSN:	0006-4971
Megjegyzések:	Coagulation factor XIII (FXIII) is a heterotetramer consisting of two catalytic A subunits (FXIII-A2) and two protective/inhibitory B subunits (FXIII-B2). FXIII-B, a mosaic protein consisting of ten sushi domains, significantly prolongs the lifespan of catalytic subunits in the circulation and prevents their slow progressive activation in plasmatic conditions. In this study the biochemistry of the interaction between the two FXIII subunits was investigated. Using surface plasmon resonance technique and an ELISA-type binding assay the equilibrium dissociation constant (Kd) for the interaction was established in the range of 10-10 M. Based on the measured Kd, it was calculated that in plasma approximately 1% of FXIII-A2 should be in free form. This value was confirmed experimentally by measuring FXIII-A2 in plasma samples immuno-depleted of FXIII-A2B2. Free plasma FXIII-A2 became functionally active, when activated by thrombin and Ca2+, it cross-linked fibrin. In cerebrospinal fluid and tears with much lower FXIII subunit concentrations higher than 80% of FXIII-A2 existed in free form. A monoclonal anti-FXIII-B antibody that prevented the interaction between the two subunits reacted with the recombinant combined 1st and 2nd sushi domains of FXIII-B and its epitope was localized to the peptide spanning positions 96-103 in the 2nd sushi domain.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban factor XII Doktori iskola
Megjelenés:	Blood. - 123 : 11 (2014), p. 1757-1763. -
További szerzők:	Pénzes-Daku Krisztina (1978-) (biológus) Csapó Andrea Fazakas Ferenc (1969-) (molekuláris biológus) Udvardy Miklós László (1977-) (orvos, tudományos segédmunkatárs) Bagoly Zsuzsa (1978-) (orvos) Orosz Zsuzsanna Zita (1982-) (orvos) Muszbek László (1942-) (haematológus, kutató orvos)
Pályázati támogatás:	TÁMOP-4.2.2/B-10/1-2010-0024 TÁMOP Laki Kálmán Doktori Iskola TÁMOP-4.2.2.A-11/1/KONV-2012-0045 TÁMOP Trombózis Kutató Központ Kutatócsoport MTA11003 MTA TKI227 MTA CNK80776 OTKA K109543 OTKA
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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