Factor XIII: Inherited and acquired deficiency

Board, P.G.; Losowsky, M. S.; Miloszewski, K.

doi:10.1016/0268-960x(93)90010-2

Cited by 190 publications

(198 citation statements)

References 127 publications

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“…3b and Table II). Three mutations, two misssense and a premature stop codon, in the ␤-barrel 2 of factor XIII A subunit were also reported to diminish enzyme activity and protein levels (61)(62)(63). Previous experiments in which deletion constructs of TGK were expressed in bacteria showed that removal of amino acids 675-816 resulted in a substantially reduced specific activity (22).…”

Section: Discussionmentioning

confidence: 99%

Consequences of Seven Novel Mutations on the Expression and Structure of Keratinocyte Transglutaminase

Huber¹,

Yee²,

Burri³

et al. 1997

Journal of Biological Chemistry

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Section: Discussionmentioning

confidence: 99%

Consequences of Seven Novel Mutations on the Expression and Structure of Keratinocyte Transglutaminase

Huber¹,

Yee²,

Burri³

et al. 1997

Journal of Biological Chemistry

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“…When activated, Factor XIII loses its 2 B carrier proteins (Board et al, 1993). It plays a pivotal role in promoting clot stability.…”

Section: Factor XIIImentioning

confidence: 99%

Cryoprecipitate transfusion: assessing appropriateness and dosing in trauma

Nascimento¹,

Rizoli²,

Rubenfeld³

et al. 2011

Transfusion Medicine

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Cryoprecipitate is commonly used outside guidelines. In trauma, the appropriate cryoprecipitate dose and its impact on plasma fibrinogen levels are unclear.This retrospective study aims to evaluate: (1) the appropriateness of cryoprecipitate transfusion in trauma; and (2) the plasma fibrinogen response to cryoprecipitate transfusion during massive transfusion in trauma.Fibrinogen levels of < 1.0 g/L within 2 and 6 hours of cryoprecipitate transfusion were used for assessing appropriateness. Out of 394 events, 238 (60%) and 259 (66%) were considered appropriate using 2 and 6 hour criteria, respectively. A dose of 8.7 (±1.7) units caused a mean increase in fibrinogen levels of 0.55 (±0.24) g/L, or 0.06g/L per unit.In our hospital, where transfusion guidelines and policies for rapid blood product and laboratory turnaround times exist, it is possible to achieve high rates of appropriateness for cryoprecipitate transfusion in trauma. The current recommended dose causes a modest increase in fibrinogen levels.iii

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“…Human Factor XIII deficiency is characterized by a life-long severe bleeding disorder, with intracranial hemorrhage (2) and recurrent abortions (3) being a significant risk. Factor XIII subunit A−/− mice have a similar phenotype with impaired clot formation and reduced clot stability (4), and also suffer from spontaneous miscarriages due to uterine bleeding (5).…”

Section: Roles Of Factor Xiiia In Hemostasis and Thrombosismentioning

confidence: 99%

Roles of transglutaminases in cardiac and vascular diseases

Sane

Kontos²,

Greenberg³

2007

Front Biosci

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All transglutaminases share the common enzymatic activity of transamidation, or the cross-linking of glutamine and lysine residues to form N epsilon (gamma-glutamyl) lysyl isopeptide bonds. The plasma proenzyme factor XIII is responsible for stabilizing the fibrin clot against physical and fibrinolytic disruption. Another member of the transglutaminase family, tissue transglutaminase or TG2 is abundantly expressed in cardiomyocytes, vascular cells and macrophages. The transglutaminases have a variety of functions independent of their transamidating activity. For example, TG2 binds and hydrolyzes GTP, thereby fostering signal transduction by several G protein coupled receptors. Accumulating evidence points to novel roles for factor XIII and TG2 in cardiovascular biology including: (a) modulating platelet activity, (b) regulating glucose control, (c) contributing to the development of hypertension, (d) influencing the progression of atherosclerosis, (e) regulating vascular permeability and angiogenesis (f) and contributing to myocardial signaling, contractile activity and ischemia/reperfusion injury. In this review, we summarize the cardiovascular biology of two members of the family of transglutaminases, Factor XIII and TG2.

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Factor XIII: Inherited and acquired deficiency

Cited by 190 publications

References 127 publications

Consequences of Seven Novel Mutations on the Expression and Structure of Keratinocyte Transglutaminase

Consequences of Seven Novel Mutations on the Expression and Structure of Keratinocyte Transglutaminase

Cryoprecipitate transfusion: assessing appropriateness and dosing in trauma

Roles of transglutaminases in cardiac and vascular diseases

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