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🩸Hema🎀 > 6: Coagulation System Part I > Flashcards

6: Coagulation System Part I Flashcards

1
Q

Is the primary substrate of thrombin

A

Factor I: Fibrinogen

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2
Q

Is the primary enzyme of the coagulation system

A

Factor I: Fibrinogen

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3
Q

Is the most concentrated of all the plasma procoagulants

A

Factor I: Fibrinogen

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4
Q

Acute phase reactants:

A
  1. Fibrinogen
  2. Prothrombin
  3. Factor VIII
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5
Q

six N-terminals assemble to form a bulky central region

A

E domain

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6
Q

three carboxyl terminals on each outer end of the molecule

A

Two D domains

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7
Q

Is the main enzyme of the coagulation pathway with multiple key activities

A

Factor II: Prothrombin

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8
Q

Activated form of factor II

A

Thrombin

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9
Q

What is the primary function of Factor II

A

Cleave fibrinopeptides (FP) A and B from the alpha and beta chains of the fibrinogen molecule

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10
Q

Functions of thrombin

A

● Activates cofactors V and VIII and factor XI by a positive feedback mechanism
● Activates factor XIII
● Initiates platelet aggregation
● Activates the protein C pathway

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11
Q

Thrombomodulin and thrombin activates

A

Protein C

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12
Q

in Thrombomodulin-Thrombin Complex, Thrombin loses its procoagulant ability to activate factors

A

factors V and VIII

because Activation of Protein C: destroys FV and FVIII

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13
Q

Thrombin-thrombomodulin also activates

A

TAFI

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14
Q

Vitamin K-Dependent Prothrombin Group

A

Procoagulants:
Prothrombin II
VII
IX
X

Regulatory proteins:
Protein C
Protein S
Protein Z
(Only protein S and Z are cofactors)

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15
Q

Clotting factor Located on membranes of fibroblasts and smooth muscle cells

A

Factor III: Tissue Factor

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16
Q

Under normal conditions, this is not expressed on blood vessel cells

A

Factor III: Tissue Factor

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17
Q

In injury, exposure of _____ leads to the activation of coagulation through VIIa

A

Factor III: Tissue Factor

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18
Q

Is required for the coagulation complexed that assemble on platelet or cell membrane phospholipids

A

Factor IV: Ionized Calcium

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19
Q

Is a glycoprotein circulating in plasma and also present in platelet alpha granules

A

Factor V: Proaccelerin

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20
Q

is a cofactor to Xa in the prothrombinase complex in coagulation

A

Factor Va

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21
Q

Accelerates thrombin generation more than 300,000-fold compared with Xa alone

A

Prothrombinase Complex

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22
Q

Is produced primarily by hepatocytes but also by microvascular ECs in lung and other tissues

A

Factor VIII: Antihemophilic Factor

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23
Q

Free form is unstable in plasma (circulates bound to vWF)

A

Factor VIII: Antihemophilic Factor

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24
Q

Explain what happens to FVIII during coagulation:

A
  1. During coagulation, thrombin cleaves FVIII from vWF and activates FVIII
  2. FVIIIa binds to activated platelets and forms the intrinsic tenase complex with factor IXa and Ca2+
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25
Is a large multimeric glycoprotein that participates in platelet adhesion
Von Willebrand Factor
26
Four sites of vWF:
1. For GP Ib/IX/V (platelet surface receptor–adhesion) 2. For GP IIb/IIIa (platelet surface receptor–aggregation) 3. Binds collagen 4. Binds factor VIII
27
ADAMTS-13 meaning
A disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13
28
Degrades vWF into smaller multimers
ADAMTS-13
29
governed by genes carried on the X chromosome
Factor VIII and Factor IX
30
Activated by the extrinsic tenase
Factor IX: Christmas Factor
31
Forms the intrinsic tenase complex with Factor VIII
Factor IX: Christmas Factor
32
Activated by the extrinsic tenase
Factor IX: Christmas Factor Factor X: Stuart-Prower Factor
33
Forms the prothrombinase complex together with Factor V
Factor X: Stuart-Prower Factor
34
Is activated by the contact factor complex
Factor XI: Plasma Thromboplastin Antecedent
35
Clotting factor more significantly activated by thrombin
Factor XI: Plasma Thromboplastin Antecedent
36
Clotting factor that activates Factor IX
Factor XI: Plasma Thromboplastin Antecedent
37
Contact Factor Complex is consists of:
Factor XII, HMWK, and Pre-K
38
they are activated by contact with negatively charged foreign surfaces
Contact Factor Complex
39
Complex that activates factor XI
Contact Factor Complex
40
Activation of the Contact Factor Complex:
1. Factor XIIa transforms pre-K into its active form, Kallikrein 2. Kallikrein cleaves HMWK to bradykinin
41
Clotting factor XII: Hageman Factor is activated in vitro by negatively charged surfaces such as:
1. Non-siliconized glass 2. Kaolin 3. Ellagic acid
42
Clotting factor XII: Hageman Factor is activated in vivo by:
1. Stents 2. Valve prostheses 3. Bacterial cell membranes
43
Factor XIII is a heterodimer; Alpha subunit is produced mostly by _____________ Beta subunit is produced in the _________
Alpha - megakaryocytes and monocytes Beta - liver
44
Covalently cross-links fibrin polymers to form a stable insoluble fibrin clot
Factor XIII: Fibrin-Stabilizing Factor
45
Is a transglutaminase that catalyzes the formation of covalent bonds between the carboxyl terminals of y chains from adjacent D domains in the fibrin polymer
Factor XIII: Fibrin-Stabilizing Factor
46
Extrinsic tenase Components:
VIIa, tissue factor, phospholipid, and Ca2+
47
Extrinsic tenase activates:
IX and X
48
Intrinsic tenase Components:
IXa, VIIIa, phospholipids, and Ca2+
49
Intrinsic tenase activates:
Factor X more efficiently
50
Prothrombinase components:
Xa, Va, phospholipids, and Ca2+
51
Prothrombinase converts:
prothrombin to thrombin
52
INTRINSIC PATHWAY
Factor XII, pre-K, HMWK, XI, IX, VIII, X, V, II, I
53
EXTRINSIC PATHWAY
Factors VII, III, X, V, II, I
54
COMMON PATHWAY
Factors X, V, II, and I
55
Normal physiologic coagulation requires the presence of two cell types for formation of coagulation complexes:
1. Cells that express TF (usually extravascular) 2. Platelets (intravascular)
56
Coagulation can be described as occurring in two phases:
1. Initiation 2. Propagation
57
occurs on tissue factor-expressing cells (produces 3%-5% of the total thrombin)
Initiation
58
occurs on platelets (produces 95% or more of the total thrombin)
Propagation
59
Provide a surface for formation and amplification of intrinsic tenase and prothrombinase complexes
COAT Platelets
60
Principal Regulators:
1. TFPI 2. Antithrombin 3. Activated Protein C
61
Is a Kunitz-type serine protease inhibitor
Tissue Factor Pathway Inhibitor
62
Is the principal regulator of the TF pathway
Tissue Factor Pathway Inhibitor
63
Kunitz of TFPI: binds to and inhibits factor Xa
Kunitz-2 domain
64
Kunitz of TFPI: binds to and inhibits the VIIa:TF complex
Kunitz-1 domain
65
cofactor of APC and TFPI; enhances factor Xa inhibition by TFPI tenfold
Protein S
66
In intact normal vessels, thrombin avidly binds the EC membrane protein thrombomodulin and triggers the
Protein C Pathway
67
Is a transmembrane protein that binds protein C adjacent to the thrombomodulin-thrombin complex
EPCR
68
augments the action of thrombin-thrombomodulin at least 5-fold in activating protein C to the serine protease APC
EPCR
69
Cofactor that binds and stabilizes APC
Protein S:
70
Protein S Is synthesized in the liver and circulates in the plasma in two forms:
1. Free protein S (40%), 2. Bound protein S (60%)
71
is covalently bound to C4bBP; cannot participate in the protein C anticoagulant pathway
Bound protein S
72
Was the first of the coagulation regulatory proteins to be identified
Antithrombin
73
Is a serine protease inhibitor (serpin) that binds and neutralizes serine proteases
Antithrombin
74
Requires heparin for effective anticoagulant activity
Antithrombin
75
an inhibitor that primarily targets thrombin
Heparin cofactor II (HC II)
76
Is available from endothelium-associated mast cell granules or as EC heparan sulfate
Heparin
77
Laboratory measurement of _______ is used as an indicator of thrombin generation.
Turnaround time (TAT)
78
is a potent inhibitor of factor Xa
Protein Z-dependent Protease Inhibitor (ZPI)
79
Also inhibits Factor XIa (does not require protein Z)
Protein Z-dependent Protease Inhibitor (ZPI)
80
Inhibition of factor XIa is accelerated by two-fold in the presence of heparin
Protein Z-dependent Protease Inhibitor (ZPI)
81
A nonspecific, heparin-binding serpin that inhibits a variety of proteases (APC, thrombin, factor Xa, factor XIa, & urokinase)
Protein C Inhibitor
82

🩸Hema🎀 (20 decks)

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