2nd International Factor XIII Workshop PROGRAM, GENERAL OVERVIEW SEPTEMBER 28, WEDNESDAY ARRIVAL REGISTRATION IN THE HOTEL 12:00-22:00 Welcome drink, dinner 19:00SEPTEMBER 29, THURSDAY REGISTRATION IN THE HOTEL 8:00-9:00 OPENING; 9:00-9:30 SCIENTIFIC PROGRAM 9:30-12:45 Lunch break 12:45-15:00 SCIENTIFIC PROGRAM 15:00-17:40 Dinner 19:00 SEPTEMBER 30, FRIDAY SCIENTIFIC PROGRAM 9:00-11:50 Lunch break 11:50-14:00 INDUSTRIAL SESSION 14:00-15:00 Excursion 16:00OCTOBER 1, SATURDAY SCIENTIFIC PROGRAM 9:00-11:50 Lunch break 11:50-13:00 SCIENTIFIC PROGRAM 13:00-17:10 Farewell dinner 18:00 OCTOBER 2, SUNDAY DEPARTURE

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DAY 1 (SEPTEMBER 29, THURSDAY) OPENING; 9:00-9:30 GREETING BY GÁBOR PAPP THE MAYOR OF HÉVÍZ GREETING BY THE CHAIRMAN OF THE ORGANIZING COMMITTEE, GENERAL INSTRUCTIONS

SCIENTIFIC PROGRAM TOPIC 1: FXIII STRUCTURE AND ACTIVATION Chairpersons: Imhof D (Germany), Kolev K (Hungary) 9:30-9:45

T1/1 REVISITING THE MECHANISM OF COAGULATION FACTOR XIII ACTIVATION AND ITS REGULATION FROM A STRUCTURE/FUNCTIONAL PERSPECTIVE Gupta S (Germany), Biswas A Germany), Krettler C (Germany), Reinhart C (Germany), Dodt J (Germany), Philippou H (UK), Ivaskevicius V (Germany), Oldenburg J (Germany)

9:45-10:05

T1/2 THE FIRST EVENTS IN BLOOD COAGULATION FXIII-A2 ACTIVATION; MODELING THE THROMBIN-FXIII-A2 COMPLEX AND THE EFFECT OF Ca2+ IONS ON THE DYNAMICS OF FXIII-A2’ Fekete A, Muszbek L, Komáromi I (Hungary)

10:05-10:20

T1/3 MASS-SPECTROMETRY STUDY OF NON-OXIDIZED AND OXIDIZED HUMAN PLASMA FIBRIN-STABILIZING FACTOR Bychkova AV, Vasylieva AD, Danilova TA, Shchegolikhin AN, Bugrova AE, Kononikhin AS, Indeykina MI, Nikolaev EN, and Rosenfeld MA (Russia)

10:20-10:35

T1/4 THE TRANSGLUTAMINASE REACTION OF BLOOD COAGULATION FACTOR XIII; A MODEL FOR THE FIRST (ACYL-ENZYME FORMATION) STEP BASED ON HYBRID QM/MM CALCULATIONS Balogh G and Komáromi I (Hungary)

10:35-10:50

T1/5 EXPRESSION ANALYSIS REVEALS VARYING DEGREES OF EFFECTIVENESS FOR HETEROZYGOUS MISSENSE MUTATIONS REPORTED FROM PATIENTS WITH MILD FXIII DEFICIENCY

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Biswas A (Germany), Thomas A (Germany), Dodt J (Germany), Philippou H (UK), Hethershaw E (Germany), Juergen HE (Germany), Ivaskevicius V (Germany), Oldenburg J (Germany) 10:50-11:15

Coffee break

TOPIC 2: INHIBITION OF FXIIIa, FXIII AND FIBRINOLYSIS Chairpersons: Pease RJ (UK), Bagoly Z (Hungary) 11:15-11:30

T2/1 TARGETED SYNTHESIS AND CHARACTERIZATION OF DIFFERENT ISOMERS OF FACTOR XIIIA INHIBITOR TRIDEGIN Bäuml CA, Loef I, Steinmetzer T, Biswas A, and Imhof D (Germany)

11:30-11:55

T2/2 STRUCTURE-ACTIVITY RELATIONSHIP STUDIES OF DISULFIDE ISOMERS OF THE FACTOR XIIIA INHIBITOR TRIDEGIN Bäuml CA, Loef I, Hardes K, Steinmetzer T, Biswas A, and Imhof D (Germany)

11:55-12:20

T2/3 COAGULATION CHANGES IN DIABETES WITH EMPHASIS ON FIBRIN AND FIBRINOLYSIS Grant PJ (UK)

12:20-12:45

T2/4 FIBRINOLYSIS: THE MAIN ENEMY OF FACTOR XIII Kolev K (Hungary)

12:45-15.00

Lunch break, visiting the lake

TOPIC 3: CELLULAR FXIII Chairpersons: Wolberg A (USA), Grant P (UK) 15:00-15:25

T3/1 RESIDENT TISSUE MACROPHAGES SECRETE PLASMA FXIIIA AND MAINTAIN THE PLASMA POOL Beckers CML (UK), Simpson KR (UK), Griffin KJ (UK), Brown JM (UK), Cordell PA (UK), Smith KA (UK), Kearney MT (UK), Vacher J (Canada), Alexander W (Australia), Grant PJ (UK) and Pease RJ (UK)

15:25-15:45

T3/2 TRANSGLUTAMINASES IN HUMAN CORNEA AND LENS Muszbek L, Orosz ZZ, Bárdos H, Shemirani AH, Vereb G, Hidasi V, Nagy B, Kappelmayer J, Ádány R, Berta A, Facskó A (Hungary)

15:45-16:00

T3/3 3

FACTOR XIII IN TEARS AND ITS POSSIBLE ROLE IN CORNEAL WOUND HEALING Orosz ZZ, Szöőr Á, Veréb Z, Hassan Z, Katona É, Vereb G, Facskó A, Muszbek L (Hungary) 16:00-16:25

Tea break

TOPIC 4: FXIII: METHODOLOGICAL ASPECTS Chairpersons: Menegatti M (Italy), Kappelmayer J (Hungary) 16:25-16:45

T4/1 THE ACTION OF FXIII MADE VISIBLE IN A MICROVASCULAR FLOW MODEL Jenny L, Kohler HP, Schroeder V (Switzerland)

16:45-17:10

T4/2 FACTOR XIII LABORATORY TESTING: LESSONS FROM THE ECAT EQA PROGRAMME Meijer P (The Netherlands)

17:10-17:25

T4/3 CORRELATION OF FXIII ACTIVITY WITH LABORATORY PARAMETERS THAT ASSESS THROMBIN GENERATION AND CLINICAL PARAMETERS IN HEMOPHILIA A PATIENTS Milos M, Coen Herak D, Zupancic-Salek S, Zadro R (Croatia)

17:25-17:40

T4/4 MONOCLONAL ANTIBODY DIRECTED AGAINST THE CROSSLINKED FIBRIN NEOEPITOPE; “DD-XLINK-MAB” Pasternack R (Germany)

19:00-

Dinner over the lake

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DAY 2 (SEPTEMBER 30, FRIDAY) TOPIC 5: FUNCTION OF FXIII Chairpersons: Ariens RAS (UK), Pieters M (South Africa) 9:00-9:25

T5/1 PLASMA FXIII, BUT NOT PLATELET FXIII, PROMOTES RED BLOOD CELL RETENTION IN CONTRACTED CLOTS Kattula S (USA), Byrnes JR (USA), Bagoly Z (Hungary), Muszbek L (Hungary), and Wolberg AS (USA)

9:25-9:50

T5/2 FACTOR XIII ACTIVITY PLAYS A ROLE IN THE RATE OF FIBRIN FORMATION Ali M (UK), Pease R (UK), Hardy L (UK), Howell G (UK), Law G (UK), La Corte AC (UK), Hethershaw E (UK), Duval C (UK), Ridger V (UK), Weisel JW (USA), Grant PJ (UK), Ariëns RAS (UK), Philippou H (UK)

9:50-10:15

T5/3 FACTOR XIII ACTIVATION PEPTIDE – A SHORT PEPTIDE WITH MULTIPLE FUNCTIONS. Kohler HP (Switzerland)

10:15-11:45

Coffee break

10:45-11:00

T5/4 FACTOR XIII AND VASCULAR SMOOTH MUSCLE CELLS Bogáti R, Katona É, Balogh E, Jeney V, Muszbek L (Hungary)

11:00-11:20

T5/5 FXIII-A AND TRANSGLUTAMINASE 2 JOINTLY PROTECT THE MYOCARDIUM AGAINST FIBROSIS Beckers CM, Newell LM, Drinkhill MJ, Griffin KJ, Cheah L, Simpson KR, Brown JM, Jackson CL, Pease RJ and Grant PJ (UK)

11:20-11:35

T5/6 FACTOR XIII DEFICIENCY ENHANCES THROMBIN GENERATION DUE TO IMPAIRED FIBRIN POLYMERIZATION - AN EFFECT CORRECTED BY FACTOR XIII REPLACEMENT Pitkänen H H, Jouppila A, Lemponen M, Ilmakunnas M, Ahonen J, Lassila R (Finland)

11:35-11:50

T5/7 PROLONGED WOUND HEALING DUE TO MODERATE FXIII DEFICIENCY IN A PATIENT WITH FEMORAL FRACTURE Bronic A (Croatia)

11:50-14.00

Lunch break, visiting the lake

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INDUSTRIAL SESSION; 14:00-15:00 Chairperson: Muszbek L (Hungary) OVERVIEW ON rFXIII AND THE CLINICAL PROGRAM

Driessler F Novo Nordisk Health Care AG, Zürich, Switzerland 16:00-

Boat excursion on the Lake Balaton

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DAY 3 (OCTOBER 1, SATURDAY) TOPIC 6: FXIII DEFICIENCY Chairpersons: Köhler HP (Switzerland), Meijer P (The Netherlands) 9:00-9:25

T6/1 CONGENITAL FACTOR XIII DEFICIENCY – UNRESOLVED PROBLEMS REGARDING NOMENCLATURE, DIAGNOSIS, AND THERAPY Schroeder V (Switzerland)

9:25-9:40

T6/2 IRANIAN COHORT, FIRST REPORT OF THE FACTOR XIII DEFICIENCY IN SISTAN AND BALOUCHESTAN PROVINCE OF IRAN Naderi M, Hosseini S, Eshghi P, Malek F, Dorgalaleh A (Iran)

9:40-9:55

T6/3 CLINICAL AND MOLECULAR CHARACTERISTICS OF PATIENTS AFFECTED BY CONGENITAL FXIII DEFICIENCY IN PAKISTAN Borhany M, Fatima N, Abid M, Shamsi TS (Pakistan)

9:55:10:10

T6/4 PROSPECTIVE EVALUATION OF BLEEDING INCIDENCE IN FACTOR XIII DEFICIENCY (PRO-RBDD STUDY) Palla R (Italy), Menegatti M (Italy), Boscarino M (Italy), Halimeh S (Germany), Lachamann B (Germany), Borhany M (Pakistan), Naveena F (Pakistan), Ozdemir N (Turkey), Siboni SM (Italy), Mikovic D (Serbia), Saracevic M (Serbia), Mumford A (UK), Harvey A (UK), Schutgens REG (The Netherland), van Haaften-Spoor M (The Netherland), Chapin J (USA), Hsu F (USA), Platokouki H (Greece), Pergantou H (Greece), Yilmaz A (Turkey), Shapiro AD (USA), Williamson A (USA), De Moerloose P (Switzerland), Casini A (Switzerland), Payne J (UK), Muszbek L (Hungary), Peyvandi F (Italy)

10:10-10:30

T6/5 MINIMUM FACTOR XIII CLOTTING LEVEL TO PREVENT MAJOR SPONTANEOUS BLEEDINGS: RESULTS FROM THE PROSPECTIVE DATABASE ON FACTOR XIII DEFICIENCY (PRO-RBDD) Menegatti M (Italy), Palla R (Italy), Boscarino M (Italy), Halimeh S (Germany), Lachamann B (Germany), Borhany M (Pakistan), Naveena F (Pakistan), Ozdemir N (Turkey), Siboni SM (Italy), Mikovic D (Serbia), Saracevic M (Serbia), Mumford A (UK), Harvey A (UK), Schutgens REG (The Netherland), van Haaften-Spoor M (The Netherland), Chapin J (USA), Hsu F (USA), Platokouki H (Greece),

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Pergantou H (Greece), Yilmaz A (Turkey), Shapiro AD (USA), Williamson A (USA), De Moerloose P (Switzerland), Casini A (Switzerland), Payne J (UK), Muszbek L (Hungary), Peyvandi F (Italy) 10:30-10:55

Coffee break

10:55-11:10

T6/6 RECOMBINANT FXIII FOR PROPHYLAXIS AND TREATMENT IN CONGENITAL FXIII DEFICIENCY: A CASE REPORT Schlammadinger Á, Árokszállási A, Kerényi A, Katona É, Bereczky Zs, Muszbek L, Boda Z (Hungary)

11:10-11:25

T6/7 BLEEDING EPISODES IN HETEROZYGOTES OF FACTOR XIII DEFICIENCY Naderi M, Eshghi P, Malek M, Karbasian F, Dorgalaleh A (Iran)

11:25-11:50

T6/8 THE DIAGNOSIS AND CHARACTERIZATION OF ALLO-, OR AUTOANTIBODIES AGAINST FACTOR XIII SUBUNITS Muszbek L (Hungary), Pénzes K (Hungary), Katona É Hungary), Bereczky Z (Hungary), Kerényi A (Hungary), Rázsó K (Hungary), Kun M (Hungary), Boda Z (Hungary), Vezina C (Canada), Rivard GE (Canada)

11:50-13:00

Lunch break

TOPIC 7: FXIII POLYMORHISMS Chairpersons: Schroeder V (Switzerland), Philippou H (UK) 13:00-13:15

T7/1 REGULATION OF PLASMA FXIII ANTIGEN LEVELS IN HEALTHY INDIVIDUALS Katona É, Mezei ZA, Kállay J, Bereczky Z, Kovács B, Ajzner É, Muszbek L (Hungary)

13:15-13:40

T7/2 FUNCTIONAL VARIANTS OF FXIII - WHAT HAVE WE LEARNED SO FAR Ariëns RAS (UK)

13:40-14:05

T7/3 THE INFLUENCE OF THE FXIII VAL34LEU POLYMORPHISM ON PLASMA CLOT STRUCTURE IN AFRICANS Pieters M (South Africa)

14:05-14:20

T7/4

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GENDER SPECIFIC EFFECT OF THE FXIII-A VAL34LEU POLYMORPHISM ON THE RISK FOR CHILDHOOD AND PERINATAL ARTERIAL ISCHEMIC STROKE Coen Herak D, Ceri A, Grzunov A, Lenicek Krleza J, Radic Antolic M, Horvat I, Djuranovic V, Barisic Nina, Zrinski Topic R, Zadro R (Croatia) 14:20-14:35

T7/5 THE EFFECT OF FXIII LEVELS AND FXIII-B POLYMORPHISMS ON THE RISK OF VENOUS THROMBOEMBOLISM Mezei ZA, Katona É, Kállay J, Bereczky Z, Somodi L, Kovács B, Miklós T, Ajzner É, Muszbek L (Hungary)

14:35-14:50

T7/6 EFFECT OF FACTOR XIII LEVELS AND POLYMORPHISMS ON THE RISK OF MYOCARDIAL INFARCTION IN YOUNG PATIENTS Bereczky Z, Mezei ZA, Balogh L, Katona É, Fiatal S, Ádány R, Miklós T, Kovács B, Ajzner É, Édes I, Muszbek L (Hungary)

14:50-15:20

Tea break

TOPIC 8: FXIII IN DISEASES Chairpersons: Bereczky Z (Hungary), Biswas A (Germany) 15:20-15:35

T8/1 FXIII LEVELS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME Bagoly Z, Tóth NK, Tarr T, Soltész P, Katona E, Mezei ZA, Muszbek L (Hungary)

15:35-15:50

T8/2 THE EFFECT OF HEMODIAFILTRATION AND HEMODIALYSIS ON COAGULATION PARAMETERS IN PATIENTS WITH ENDSTAGE RENAL DISEASE Pénzes K, Becs G, Hurják B, Molnár E, Katona É, Balla J and Muszbek L (Hungary)

15:50-16:15

T8/3 INTRACELLULAR FACTOR XIII-A IS A FLOW CYTOMETRIC DIAGNOSTIC TEST IN ACUTE LEUKEMIAS Kappelmayer J (Hungary)

16:15-16:30

T8/4 FACTOR XIII-A POSITIVE MACROPHAGES AND CD11c POSITIVE DENDRITIC CELLS IN MALIGNANT MELANOMA Bárdos H, Töröcsik D, Emri E, Dezső B, Emri G, Remenyik E, Balázs M , Ádány R (Hungary)

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16:30-16:45

T8/5 EXPRESSION OF BLOOD COAGULATION FACTOR XIII-A IN LYMPHOBLASTS PREDICTS FAVOURABLE OUTCOME IN PEDIATRIC PRECURSOR B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA Kárai B, Szánthó E, Csáthy L, Ujfalusi A, Gyurina K, Szegedi I, Kappelmayer J, Kiss Cs, Hevessy Zs (Hungary)

16:45-17:10

T8/6 IMMUNOHISTOCHEMICAL DETECTION OF FACTOR XIII SUBUNIT A AS A DIAGNOSTIC TOOL IN DERMATOPATHOLOGY: BELIEFS AND DISBELIEFS Törőcsik D, Bárdos H, Ádány R (Hungary)

18:00-

Visiting the Festetics Palace in Keszthely and farewell dinner

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