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Titolo:
Gene targeting in hemostasis. Factor XI
Autore:
Gailani, D;
Indirizzi:
Vanderbilt Univ, Div Hematol Oncol, Dept Pathol, Nashville, TN 37232 USA Vanderbilt Univ Nashville TN USA 37232 pt Pathol, Nashville, TN 37232 USA Vanderbilt Univ, Div Hematol Oncol, Dept Med, Nashville, TN 37232 USA Vanderbilt Univ Nashville TN USA 37232 Dept Med, Nashville, TN 37232 USA
Titolo Testata:
FRONTIERS IN BIOSCIENCE
, volume: 6, anno: 2001,
pagine: D201 - NIL_1
SICI:
1093-9946(20010201)6:<D201:GTIHFX>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
BLOOD-COAGULATION FACTOR; MOLECULAR-WEIGHT KININOGEN; HUMAN-PLASMA PREKALLIKREIN; ACTIVATED FACTOR-XI; 4 TANDEM REPEATS; BINDING-SITE; FACTOR-IX; APPLE DOMAINS; PROTEIN-C; DISULFIDE BONDS;
Keywords:
gene targeting; hemostasis; thrombosis; factor XI; review;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
60
Recensione:
Indirizzi per estratti:
Indirizzo: Gailani, D Vanderbilt Univ, Div Hematol Oncol, Dept Pathol, 538 MRB II,2220 Pierce Ave, Nashville, TN 37232 USA Vanderbilt Univ 538 MRB II,2220 Pierce Ave Nashville TN USA 37232
Citazione:
D. Gailani, "Gene targeting in hemostasis. Factor XI", FRONT BIOSC, 6, 2001, pp. D201-NIL_1

Abstract

Factor XI (FXI) is the zymogen of a plasma serine protease (FXIa) that contributes to hemostasis by activating factor IX (FIX). This reaction appearsto be important for sustaining thrombin production after initial fibrin formation, to consolidate and protect fibrin clots from degradation by fibrinolysis. Humans with congenital FXI deficiency have a variable propensity tobleed after trauma or surgery, but do not experience the "spontaneous" hemorrhage in joints and soft tissue characteristic of hemophilia (FVIII or FIX deficiency). Mice homozygous for a disruption of the FXI gene (FXI-/-) have prolonged activated partial thromboplastin times and no detectable plasma FXI activity. Like their human counterparts, FXI-/- animals are generallyhealthy, reproduce normally, and do not develop spontaneous hemorrhage. Intail bleeding time assays, FXI-/- animals may have slightly prolonged bleeding compared to FXI+/+ and FXI+/- animals, however, a consistent hemostatic deficit has not been identified. More impressive results are obtained when FXI-/- mice are crossed with protein C deficient mice. Severe FXI deficiency partially ameliorates the devastating hypercoagulable state associated with severe protein C deficiency, indicating that FXI plays a role in certain thrombotic conditions.

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Documento generato il 22/01/20 alle ore 13:22:13