α1-antitrypsin and the maintenance of hemostatic balance

Haematologica

Volumn 94, Issue 6, 2009, Pages 762-763

  Emmerich, Joseph ^a  

^a UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA 1 ANTITRYPSIN; BLOOD CLOTTING FACTOR 10A; BLOOD CLOTTING FACTOR 11A; BLOOD CLOTTING FACTOR 12A; BLOOD CLOTTING FACTOR 9A; PROTEIN C; THROMBIN; ALPHA 1 ANTITRYPSIN PITTSBURGH; ALPHA 1-ANTITRYPSIN PITTSBURGH; BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 11; BLOOD CLOTTING FACTOR 12;

BLEEDING; BLEEDING DISORDER; BLOOD CLOTTING; CLINICAL FEATURE; HEMOSTASIS; HUMAN; LOW DRUG DOSE; MUTATIONAL ANALYSIS; SHORT SURVEY; THROMBIN TIME; THROMBOCYTE AGGREGATION; BLOOD; BLOOD CLOTTING TEST; GENETICS; METABOLISM; MUTATION; NOTE; NUCLEOTIDE SEQUENCE;

ALPHA 1-ANTITRYPSIN; BLOOD COAGULATION TESTS; DNA MUTATIONAL ANALYSIS; FACTOR X; FACTOR XI; FACTOR XII; HEMORRHAGIC DISORDERS; HUMANS; MUTATION;

EID: 66749105118     PISSN: 03906078     EISSN: 15928721     Source Type: Journal    
DOI: 10.3324/haematol.2009.006882     Document Type: Short Survey

References (10)

1. Law RH, Zhang Q, McGowan S, Buckle AM, Silverman GA, Wong W, et al. An overview of the serpin superfamily. Genome Biol 2006;7:216-226 (Pubitemid 44782985)
2. Ardelt W, Lakowski M. Turkey ovomucoid third domain inhibits eight different serine proteinases of varied specificity on the same Leu 18-Glu 19 reactive site. Biochemistry 1985;24:5313-5320 (Pubitemid 16228805)
3. Egeberg O. Inherited antithrombin III deficiency causing thrombophilia. Thromb Diath Haemorrhag 1965;13:516-530
4. Lewis JH, Iammarino RM, Spero JA, Hasiba U. Antithrombin Pittsburgh: an a1-antitrypsin variant causing hemorrhagic disease. Blood 1978;51:129-137
5. Owen MC, Brennan SO, Lewis JH, Carrel RW. Mutation of antitrypsin to antithrombin. A1-antitrypsin Pittsburg (358 Meth ->Arg), a fatal bleeding disorder. N Engl J Med 1983; 309:694-698
6. Vidaud D, Emmerich J, Alhenc-Gelas M, Yvart J, Fiessinger JN, Aiach M. Met 358 to Arg mutation of alpha1-antitrypsin associated with protein C deficiency in a patient with mild bleeding tendency. J Clin Invest 1992;89:1537-1543
7. Emmerich J, Alhenc-Gelas M, Gandrille S, Guichet C, Fiessinger JN, Aiach M. Mechanism of protein C deficiency in a patient with arginine 358 alpha1-antitrypsin(Pittsburg mutation): role in the maintenance of hemostatic balance. J Lab Clin Med 1995;125:531-539
8. Hua B, Fan L, Liang Y, Zhao Y, Tuddenham EGD. Alpha1- antitrypsin Pittsburg in a family with bleeding tendency. Haematologica 2009;94:881-884
9. Heeb MJ, Griffin JH. Physiologic inhibition of human activated protein C by a1 antitrypsin. J Biol Chem 1988;263: 11613-11616 (Pubitemid 18212222)
10. Heeb MJ, Bischoff R, Courtney M, Griffin JH. Inhibition of activated protein C by recombinant alpha1-antitrypsin variants with substitution of arginine or leucine for methionine 358. J Biol Chem 1990;265:2365-2369 (Pubitemid 120026535)