Thrombosis and Haemostasis, Table of Contents Thromb Haemost 1981; 46(03): 621-622DOI: 10.1055/s-0038-1653431 Original Article Schattauer GmbH Stuttgart Subunit A of Factor XIII Regulates Subunit B Plasma Concentration F Rodeghiero * The Divisione di Ematologia, Ospedale Regionale di Vicenza, Italy , M Morbin * The Divisione di Ematologia, Ospedale Regionale di Vicenza, Italy , T Barbui ** The Divisione di Ematologia, Ospedali Riuniti di Bergamo, Italy › Author Affiliations Recommend Article Abstract PDF Download Buy Article Summary Subunit a of Factor XIII is found absent in homozygotes and reduced in heterozygotes. Since a concomitant reduction of subunit b occurs in these cases, an interaction between the loci controlling the synthesis of the two subunits was suggested. However in the present study we have shown that the administration of subunit a in two totally devoid homozygotes produced an increase of subunit b, reaching the maximum concentration five days after infusion. This strongly suggests that subunit b plasma level is regulated on the subunit a plasma amount. Keywords KeywordsBlood coagulation Factor XIII - Molecular subunits - Plasma level of Factor XIII PDF (126 kb) References References 1 Schwartz ML, Pizzo SV, Hill RL, McKee PA. Human factor XIII from plasma and platelets. J Biol Chem 1973; 248: 1395-1407 2 Schwartz ML, Pizzo SV, Hill RL, McKee PA. The subunit structure of human plasma and platelet factor XIII (fibrin stabilizing factor). J Biol Chem 1971; 246: 5851-5859 3 Curtis CG, Brown KL, Credo RB, Domanik RA, Gray A, Stenberg P, Lorand L. Calcium dependent unmasking of active center cysteine during activation of fibrin stabilizing factor. Biochemistry 1974; 13: 3774-3780 4 Bohn H, Becker W, Trobisch H. Die molekulare Struktur der fibrinstabilisierenden Faktoren des Menschen. II. Vergleichende immunologische Untersuchungen von Faktor-XIII-Mangel-Plasma und Normalplasma. Blut 1973; 26: 303-311 5 Israels ED, Paraskevas F, Israels GL. Immunological studies of coagulation factor XIII. J Clin Invest 1973; 52: 2398-2403 6 Barbui T, Rodeghiero F, Dini E. Factor XIII subunits-A and -S in congenital deficiency and in acute myeloblastic leukemia. Haematologica 1974; 59: 458-466 7 Egbring R, Andrassy K, Havemann K, Fuchs G, Ruf B, Scander K, Trobisch H. Erfahrungen bei der Langzeitbehandlung des angebore-nen Faktor-XIII-Mangels mit Faktor XIII-Konzentrat. Blut 1976; 33: 367-376 8 Zimmerman R, Ehlers W, Manz F, Meinrenken W, Egbring R, Gemeke H. Ein neuer Fall von schwerem Faktor-XIII-Mangel. Untersuchungen zum Verhalten der Untereinheiten und des Turnover des fibrinstabilisierenden Faktors Blut 1977; 35: 457-464 9 Girolami A, Burul A, Fabris F, Betterle C. A tentative classification of Factor XIII deficiency in two groups. Acta haematol 1977; 58: 318-320 10 Francis JL, Todd PJ. Factor XIII deficiency. A family study by measurement of factor XIII subunits A and S Acta haematol 1979; 62: 167-172 11 Barbui T, Rodeghiero F, Dini E, Mariani G, Papa ML, De Biasi R, Cordero MurilloR, Montero Umana C. Subunit A and S inheritance in four families with congenital Factor XIII deficiency. Br J Haematol 1978; 38: 267-271 12 Barbui T, Di Luzio V. Deficienza congenita del fattore stabilizzante la fibrina (fattore XIII). Giorn Clin Med 1968; 49: 46-50 13 Lorand L, Urayama T, DeKeiwiet JW C, Nossel KL. Diagnostic and genetic studies on fibrin stabilizing factor with a new assay based on amine incorporation. J Clin Invest 1969; 48: 1054-1064 14 Rodeghiero F, Barbui T. Fibrin cross-linking in congenital Factor XIII deficiency. J Clin Path 1980; 33: 434-437 15 Credo RB, Curtis CG, Lorand L. Ca2+ related regulatory function of fibrinogen. Proc Natl Acd Sei USA 1978; 75: 4234-4237
