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Hematology 2 > Coagulation System (Part I) > Flashcards

Coagulation System (Part I) Flashcards

1
Q

Is the primary substrate of thrombin

A

Clotting Factor I: Fibrinogen

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2
Q

Is the most concentrated of all the plasma procoagulants

A

Clotting Factor I: Fibrinogen

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3
Q

Describe Fibrinogen Molecule

A

✅ Is a mirror-image dimer
✅ consist of three nonidentical polypeptides
(Aα, Bβ, y) united by disulfide bonds

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4
Q

six N-terminals assemble to form a bulky central region

A

E domain

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5
Q

three carboxyl terminals on each outer end of the molecule

A

Two D domains

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6
Q

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

A

Clotting Factor II: Prothrombin

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7
Q

The cleave fibrinogen by thrombin is called

A

fibrin monomer

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8
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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9
Q

activates Protein C

A

Thrombomodulin-Thrombin Complex

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10
Q

Thrombomodulin-Thrombin Complex

A

✅ activates Protein C
✅ Thrombin loses its procoagulant ability to activate factors V and VIII
✅ Activation of Protein C: Destroys FV and FVIII
✅ Thrombin-thrombomodulin also activates TAFI

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11
Q

The only cofactors of Vitamin K-Dependent Prothrombin Group

A

protein S and Z

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12
Q

Vitamin K Is a quinone found in green leafy vegetables and is produced by the intestinal organisms

A

Bacteroides fragilis and Escherichia coli

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13
Q

✅ des-y-carboxyl proteins
✅ Cannot participate in the coagulation reaction because they lack the second carboxyl group
✅ Results from Vitamin K deficiency or in the presence of Coumadin

A

Proteins Induced by Vitamin K Antagonists (PIVKA) factors

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14
Q

In injury, exposure of TF leads to the activation of coagulation through

A

VIIa

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15
Q

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

A

Clotting Factor IV: Ionized Calcium

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16
Q

Serine proteases bind to negatively charged phospholipid surfaces, predominantly ________ through positively charged calcium ions

A

phosphatidyl serine,

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17
Q

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

A

Clotting Factor V: Proaccelerin

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18
Q

is a cofactor to Xa in the prothrombinase complex in coagulation

A

Factor Va

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19
Q

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

A

Prothrombinase Complex

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20
Q

Exposure of tissue factor during vessel injury activates the coagulation cascade through

A

Clotting Factor VII: Proconvertin

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21
Q

Is a cofactor that circulates linked to a large carrier protein, vWF

A

Clotting Factor VIII: Antihemophilic Factor

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22
Q

are key proteins for hemostasis

A

FVIII and vWF

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23
Q

Describe During coagulation associated with FVIII

A

✅,During coagulation, thrombin cleaves FVIII from vWF and activates FVIII.

✅ FVIIIa binds to activated platelets and forms the intrinsic tenase complex with factor IXa and Ca2+

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24
Q

Is a large multimeric glycoprotein that participates in platelet adhesion

A

Von Willebrand Factor

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25
Q

Transports the procoagulant factor VIII

A

Von Willebrand Factor

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26
Q

Are stored in alpha granules in platelets and in Weibel-Palade bodies in EC

A

Von Willebrand Factor

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27
Q

Four sites of vWF:

A
  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
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28
Q

a disintegrin and metalloprotease with a
thrombospondin type 1 motif, member 13

A

ADAMTS-13

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29
Q

Degrades vWF into smaller multimers

A

ADAMTS-13

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30
Q

Activated by the extrinsic tenase

A

Clotting Factor IX: Christmas Factor
Clotting Factor X: Stuart-Prower Factor

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31
Q

Forms the intrinsic tenase complex with Factor VIII

A

Clotting Factor IX: Christmas Factor

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32
Q

Forms the prothrombinase complex together with Factor V

A

Clotting Factor X: Stuart-Prower Factor

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33
Q

Is activated by the contact factor complex

A

Clotting Factor XI: Plasma Thromboplastin
Antecedent

34
Q

More significantly activated by thrombin

A

Clotting Factor XI: Plasma Thromboplastin Antecedent

35
Q

Activates Factor IX

A

Clotting Factor XI: Plasma Thromboplastin Antecedent

36
Q

Contact Factor Complex is consists of

A

Factor XII, HMWK, and Pre-K

37
Q

Activates factor XI

A

Contact Factor Complex

38
Q

Are so named because they are activated by contact with negatively charged foreign surfaces

A

Contact Factor Complex

39
Q

Clotting Factor XII: Hageman Factor

Is activated in vitro by negatively charged surfaces such as:

A

• Non-siliconized glass
• Kaolin
• Ellagic acid

40
Q

Clotting Factor XII: Hageman Factor

Is activated in vivo by:

A

• Stents
• Valve prostheses
• Bacterial cell membranes

41
Q

Activation of the Contact Factor Complex:

A
  1. Factor XIIa transforms pre-K into its active form, Kallikrein
  2. Kallikrein cleaves HMWK to bradykinin
42
Q

● Activated by thrombin
● Covalently cross-links fibrin polymers to form a stable insoluble fibrin clot

A

Clotting Factor XIII: Fibrin-Stabilizing Factor

43
Q

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

A

Clotting Factor XIII: Fibrin-Stabilizing Factor

44
Q

Coagulation Pathway Complexes

A
  1. Intrinsic tenase
  2. Extrinsic tenase
  3. Prothrombinase
45
Q

Each Coagulation Pathway Complexes is composed of:

A
  1. Vitamin K-dependent serine protease (IX, X, VII, II)
  2. Nonenzyme cofactor (VIII, V, III)
  3. Calcium and phospholipid
46
Q

Extrinsic tenase

A

✅ FactorVII
✅ Activates Factor IX and X

47
Q

Intrinsic tenase

A

✅ Factor IXa : Factor VIII
✅ Activates Factor X more e ciently

48
Q

Prothrombinase

A

✅ Factor Xa : Factor Va
✅ Converts prothrombin to thrombin

49
Q

Intrinsic Pathway

The coagulation factors in order of reaction are

A

Factor XII, pre-K, HMWK, XI, IX, VIII, X, V, II, I

50
Q

Formation of TF:VIIa has since proven to be the primary in vivo initiation mechanism for coagulation

A

Extrinsic Pathway

51
Q

Extrinsic Pathway

Includes the following factors:

A

Factors VII, X, V, II, I

52
Q

The two pathways (intrinsic and extrinsic) have in common Factors

A

Factors X, V, II, and I

53
Q

The low levels of thrombin generated in the initiation phase:

A
  1. Activates platelets through cleavage of PAR-1 and PAR-2
  2. Activates factor V released from alpha granules
  3. Activates factor VIII and dissociates it from vWF
  4. Activates factor XI
  5. Splits fibrinogen peptides A and B
54
Q

Provide a surface for formation and amplification of intrinsic tenase and prothrombinase complexes

A

COAT platelets

55
Q

Principal Regulators

A
  1. TFPI
  2. Antithrombin
  3. Activated Protein C
56
Q

Is the principal regulator of the TF pathway

A

Tissue Factor Pathway Inhibitor

57
Q

Deserve the Kunitz-type serine proteas Tissue Factor Pathway Inhibitor

A

✅ Kunitz-2 domain: binds to and inhibits factor
Xa

✅ Kunitz-1 domain: binds to and inhibits the
VIIa:TF complex

58
Q

cofactor of APC and TFPI; enhances
factor Xa inhibition by TFPI tenfold

A

Protein S

59
Q

Cofactor that binds and stabilizes APC

A

Protein S

60
Q

Requires heparin for e ective anticoagulant activity

A

Antithrombin

61
Q

What is the available form of Antithrombin

A

Heparin

62
Q

Other Serine Protease Inhibitors

A
  1. ZPI
  2. Protein C inhibitor
  3. α1-antitrypsin
  4. α2-macroglobulin
  5. α2-antiplasmin
  6. PAI-1
63
Q

In the presence of its cofactor protein Z, is a potent inhibitor of factor Xa

A

Protein Z-dependent Protease Inhibitor (ZPI)

64
Q

Also inhibits Factor XIa. Inhibition of factor XIa is accelerated by two- fold in the presence of heparin

A

Protein Z-dependent Protease Inhibitor (ZPI)

65
Q

A nonspecific, heparin-binding serpin that inhibits a variety of proteases (APC, thrombin, factor Xa, factor XIa, & urokinase)

A

Protein C Inhibitor

66
Q

Activation of ________ - the primary step in coagulation because it could be found in blood.

A

Factor XII

67
Q

occurs on tissue factor-expressing cells

A

Initiation

68
Q

occurs on platelets

A

Propagation

69
Q

produces 95% or more of the total thrombin generated

A

Propagation

70
Q

produces 3% - 5% of the total thrombin generated

A

Initiation

71
Q

revises thrombin’s function from a procoagulant enzyme to an anticoagulant

A

Protein C Regulatory System

72
Q

first to be identified; inhibits Factor IIa, IXa, Xa, XIa, XIIa, PK, Plasmin

A

Antithrombin

73
Q

inactivates thrombin

A

Heparin cofactor II

74
Q

dependent protease inhibitor - potent inhibitor of Factor Xa; also inhibits Factor XIa

A

Protein Z-

75
Q

inhibits APC, IIa, Xa, XIa, urokinase

A

Protein C inhibitor

76
Q

Mixing studies or Substitution studies

Fresh Plasma

A

All factors are present

77
Q

Mixing studies or Substitution studies

Fresh Serum

A

Lacks Factors I, V, VIII, XIII (Fibrinogen group)

78
Q

Mixing studies or Substitution studies

Aged Plasma

A

Lacks Factors V and VIII (labile factors)

79
Q

Mixing studies or Substitution studies

Aged Serum

A

Lacks Factors I, II, V, VIII, XIII (Fibrinogen group and II)

80
Q

Mixing studies or Substitution studies

Adsorbed Plasma

A

Lacks Factors II, VII, IX, X (Vit. K-dependent group)

Hematology 2 (18 decks)

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