Chapter 37: Normal Hemostasis and Coagulation (COAGULATION SYSTEM

Study online at https://quizlet.com/_4m0gqy

16 The plasma transports at least how many procoagulants ?
Liver Nearly all procoagulants are glycoproteins synthesized in the?
How many are enzymes that circulate in an inactive form called
8
zymogens?
Are those that bind, stabilize, and enhance the activity of their
Cofactors
respective enzymes?
The International Committee for the Standardization of the They officially named the plasma procoagulants using Roman
Nomenclature of the Blood Clotting Factors numerals
Fibrinogen Factor I
Prothrombin Factor II
Was assigned to a procoagulant that later was determined to be
Factor VI
activated factor V
Antihemophilic factor Factor VIII
Factor VIII is a cofactor that circulates linked to a large carrier protein, VWF
Prekallikrein Also known as the fletcher factor
HMWK Also known as the Fitzgerald factor
The plasma procoagulants may be serine proteases or cofactors,
Factor XIII
except for ___________, which is a transglutaminase
proteolytic enzymes of the trypsin family and include the proco-
Serine protease agulants thrombin (factor IIa); factors VIIa, IXa, Xa, XIa, and XIIa;
and pre-K
are synthesized as inactive zymogens consisting of a single pep-
Serine protease
tide chain.
occurs when the zymogen is cleaved at one or more specific sites
Activation
by the action of another protease during the coagulation process
Tissue factor
Factor V The procoagulant cofactors that participate in complex formation
Factor VIII are:
HMWK
Thrombin
Factor VIIa
Factor IXa
Factor Xa Serine proteases
Factor XIa
Factor XIIa
Pre-K
Fibrinogen The ultimate substrate of the coagulation pathway
Fibrinogen Forms the primary structural protein of the fibrin clot
Forms the primary structural protein of the fibrin clot which is
Fibrinogen
further stabilized by factor XIII
Required for the assembly of coagulation complexes on platelet
Calcium
or cell membrane phospholipids
Bind to negatively charged phospholipid surfaces, predominantly
Serine protease
phosphatidylserine, through positively charged calcium ions
If zymogen activation is uncontrolled and generalized, the condi-
DIC
tion is called
Factor VIII Has a short a half-life of 12 hours
Prothrombin
Factor VII
Factor IX Vitamin-K dependent factors (prothrombin group)
Factor X
Protein C
1/5
 Chapter 37: Normal Hemostasis and Coagulation (COAGULATION SYSTEM
Study online at https://quizlet.com/_4m0gqy
Protein S
Protein Z
Is a quinone found in green leafy vegetables and is produced by
Vitamin K
the intestinal organisms Bacteroides fragilis and Escherichia coli
Bacteroides fragilis and Escherichia coli Intestinal organisms that produces Vitamin K
catalyzes an essential posttranslational modification of the pro-
Vitamin K thrombin group proteins: g-carboxylation of amino-terminal glu-
tamic acids
In this condition a vitamin K antagonist, the vitamin K-dependent
Vitamin K deficiency or in the presence of warfarin procoagulants are released from the liver without the second
carboxyl group added to the g carbon
they lack the second carboxyl group, they cannot bind to Ca21
des-y-carboxyl proteins or PIVKAs and phospholipid, so they cannot participate in the coagulation
reaction
Vitamin K antagonism Is the basis for oral anticoagulant therapy
Essential for the assembly of three membrane complexes leading
Vitamin K-dependent procoagulants
to the generation of thrombin
Is composed of factor VIIa and tissue factor, and it activates factors
Extrinsic tenase IX and X, which are components of the next two complexes,
intrinsic tenase and prothrombinase, respectively
Factor VIIa and tissue factor Extrinsic tenase is composed of?
Factors IX and X The extrinsic tenase activates what factors?
is composed of factor IXa and its cofactor VIIIa; it also activates
Intrinsic tenase
factor X much more efficiently than the TF:VIIa complex
Factor IXa and VIIIa Components of the intrinsic tenase
Factor X Activated by the intrinsic tenase
Prothrombinase Composed of factor Xa and its cofactor Va
this converts prothrombin to thrombin in a multistep hydrolytic
Prothrombinase process that releases thrombin and a peptide fragment called
prothrombin fragment 1.2
Prothrombinase converts prothrombin to thrombin in a multistep
Prothrombin fragment 1.2 hydrolytic process that releases thrombin and a peptide fragment
called?
Prothrombin fragment 1.2 in plasma A marker for thrombin generation
Tissue factor
Factor V
Procoagulant cofactors
Factor VIII
HMWK
Procoagulant cofactors tissue factor, factor V, factor VIII, and HMWK are called the?
Thombomodulin
Protein S Coagulation control cofactors
Protein Z
Coagulation control cofactors thrombomodulin, protein S, and protein Z are called the
Thrombomodulin Also a cofactor in control of fibrinolysis
is a transmembrane receptor for factor VIIa and is found on ex-
Tissue factor travascular cells such as fibroblasts and smooth muscle cells, but
under normal conditions, it is not found on blood vessel ECs.
Blood vessel ECs Tissue factor is not found on?
expressed in high levels in cells of the brain, lung, placenta, heart,
Tissue factor
kidney, and testes

2/5
Chapter 37: Normal Hemostasis and Coagulation (COAGULATION SYSTEM
Study online at https://quizlet.com/_4m0gqy
Brain
Lung
Heart
Tissue factor is expressed in high levels where?
Placenta
Kidney
Testes
Factor V and VIII Soluble plasma proteins
Factor V and VIII Both are activated by thrombin and inactivated by protein C
Thrombin Factor V and VIII are activated by?
Protein C Factor V and VIII are inactivated by?
A glycoprotein circulating in plasma and also present in platelet
Factor V
a-granules
Factor Va A cofactor to Xa in the prothrombinase complex in coagulation
Accelerates thrombin generation more than 300,000-fold com-
Prothrombinase complex
pared to Xa alone
Thrombomodulin-bound thrombin Activates protein C
Protein C Inactivates Va to Vi
is both activated and then ultimately inactivated by the generation
Factor V
of thrombin, as is factor VIII
Factor VIII A cofactor to Factor IX
a cofactor to factor XIIa and prekallikrein in the intrinsic contact
HMWK
factor complex
a transmembrane protein constitutively expressed by vascular
Thrombomodulin
ECs, is a thrombin cofactor
a coagulation regulatory protein, and thrombin activatable fibri-
Protein C
nolysis inhibitor (TAFI), a fibrinolysis inhibitor
Protein S A cofactor to protein C, as well as TFPI
Protein Z Cofactor to ZPI
Hepatocytes Factor VIII is produced primarily by?
Free factor VIII Unstable in plasma; it circulates bound to VWF
binds to activated platelets and forms the intrinsic tenase complex
Factor VIIIa
with factor IXa and Ca2+
are the two plasma procoagulants whose production is governed
Factor VIII and Factor IX
by genes carried on the X chromosome
Hemophilia A Factor VIII deficiency
Hemophilia B Factor IX deficiency
Hemophilia A Have diminished factor VIII activity but normal VWF levels
A large multimeric glycoprotein that participated in platelet adhe-
VWF
sion ad transports the procoagulant factor VIII
Group O These individuals have lower levels of VWF than other ABO types
an acute phase protein, as is factor VIII, and levels increase in
VWF
pregnancy, trauma, infections, and stress.
Contact factors Also called the intrinsic accessory pathway proteins
Factor XII
HMWK Contact factors
Pre-K
Contact factors activated by contact with negatively charged foreign surfaces
Pre-K A glycoprotein that circulates bound to HMWK
Kallikrein Cleaves HMWK to for bradykinin

3/5
Chapter 37: Normal Hemostasis and Coagulation (COAGULATION SYSTEM
Study online at https://quizlet.com/_4m0gqy
Factor XII and pre-K Are zymogens that are activated to become serine proteases
HMWK A nonenzymatic cofactor
HMWK:pre-K:FXII Contact factor complex
is activated by the contact factor complex and, more significantly,
Factor XI by thrombin during coagulation generated from tissue factor acti-
vation
Contact factor complex Activates factor XI
Factor XIa An activator of Factor IX
is activated in vitro by negatively charged surfaces such as non-
Factor XII siliconized glass, kaolin, or ellagic acid in partial thromboplastin
time (PTT) reagents
Nonsiliconized glass
Kaolin Negatively charged surfaces
Ellagic acid
In vivo, foreign materials that may activate contact factors to cause
Stents or valve prostheses
thrombosis
Rosenthal syndrome Deficiency in factor XI usually result in mild and variable bleeding
Its primary function is to cleave fibrinopeptides A and B from the
Thrombin a and b chains of the fibrinogen molecule, triggering spontaneous
fibrin polymerization
amplifies the coagulation mechanism by activating cofactors V
Thrombin
and VIII and factor XI by a positive feedback mechanism
Thrombin Also initiates aggregation of platelets
plays a role in coagulation (fibrin), in platelet activation, in coag-
Thrombin
ulation control (protein C), and in controlling fibrinolysis (TAFI)
Thrombin considered the key protease of the coagulation pathway
Fibrinogen Primary substrate of thrombin,
is also essential for platelet aggregation because it links activated
Fibrinogen
platelets through their GP IIb/IIIa platelet fibrinogen receptor
Fibrinogen is a 340,000 Dalton glycoprotein synthesized in what
Liver
organ?
200-400 mg/dL Normal plasma concentration of fibrinogen
Fibrinogen the most concentrated of all the plasma procoagulants
is an acute phase reactant protein, whose level increases in
Fibrinogen
inflammation, infection, and other stress conditions
Six N-terminals assemble to form a bulky central region called the E domain
E domain Bulky central region
Carboxyl terminals assemble at the outer ends of the molecule to form two D domains
cleaves fibrinopeptides A and B from the protruding N-termini of
Thrombin each of the two a and b chains of fibrinogen, reducing the overall
molecular weight by 10,000 Daltons
Fibrin monomer The cleaved fibrinogen is called
a heterodimer whose a subunit is produced mostly by megakary-
Factor XIII ocytes and monocytes, and whose b subunit is produced in the
liver
Megakaryocytes and monocytes The alpha subunit of factor XIII is produced mostly by?
Liver The beta subunit of factor XIII is produced in the?
covalently crosslinks fibrin polymers to form a stable insoluble
Factor XIIIa
fibrin clot

4/5
 Chapter 37: Normal Hemostasis and Coagulation (COAGULATION SYSTEM
Study online at https://quizlet.com/_4m0gqy
is a transglutaminase that catalyzes the formation of covalent
Factor XIIIa bonds between the carboxyl terminals of g chains from adjacent
D domains in the fibrin polymer
Fibronectin A plasma protein involved in cell adhesion
a2-antiplasmin Render the fibrin mesh resistant to fibrinolysis
the primary serine protease of the fibrinolytic system, also be-
Plasminogen
comes covalently bound via lysine moieties
a serine protease that ultimately hydrolyzes and activates bound
TPA
plasminogen to initiate fibrinolysis
Activation of factor XII The primary step in coagulation
the reaction system that begins with factor XII and culminates in
Intrinsic pathway
fibrin polymerization has been called the
The coagulation factors of the intrinsic pathway, in order of reac-
XII, pre-K, HMWK, XI, IX, VIII, X, V, prothrombin, and fibrinogen
tion, are:
The laboratory test that detects the absence of one or more of
APTT or PTT
these factors
Formation of TF:VIIa The primary in vivo initiation mechanism for coagulation
Extrinsic pathway Tissue factor pathway has been called the?
VII, X, V, prothrombin, and fibrinogen Factors included in the extrinsic pathway
PT The test used to measure the integrity of the extrinsic pathway
X, V, prothrombin, fibrinogen Factors involved in the common pathway
Operationally, coagulation can be described as occurring in two
Initiation and propagation
phases:
which occurs on tissue factor-expressing cells and produces 3%
Initiation
to 5% of the total thrombin generated
occurring on platelets, which produces 95% or more of the total
Propagation
thrombin
refers to extrinsic tenase complex formation and generation of
Initiation
small amounts of factor Xa, factor IXa, and thrombin
In this phase the reactions occur on the surface of the activated
Propagation platelet, which now has all the components needed for coagula-
tion
COAT platelets Platelets partially activated by collagen and thrombin

5/5