Page 1871 - Williams Hematology ( PDFDrive )

1846 Part XII: Hemostasis and Thrombosis Chapter 112: Platelet Morphology, Biochemistry, and Function 1847

TABLE 112–2. Adhesive Glycoproteins
Unusual 1° Domain Homol-
Structural Features ogies & Binding Mature Protein Mature Protein
Protein Subunit, κ Da & Modifications Regions Composition M Known Interactions
r
Collagens 95–180 Gly-Pro-X repeating RGD Tropocollagen = Variable
sequence Right-handed tri- 3 chains Thrombospondin
Hydroxylysine ple helix
Hydroxyproline
Type I α (I) DGEA † [α (I)] α (I) (major Fibronectin
1
2 2
1
α (I) VWFC component) von Willebrand factor
2
[α (I)] 3
1
Type III α (III) VWFC [α (III)] 3
1
1
Type VI α (VI) 3 VWFA α (VI)α (VI)α (VI)
2
3
1
1
α (VI) 3 VWFA
2
α (VI) 12 VWFA
3
von 220 (2050 Large propeptide (741 α β – RGD Dimer = 880,000– Collagen
IIb 3
Willebrand amino acids) amino acids); A, B, C, D, 1789–1791 I protomer Multim- ~20,000,000 Heparin
factor E repeats Domains ers of protomers Factor VIII
GPlb – 230–310 from 2–~40 via Fibrin
disulfide bonds
Fibrinogen Aα = 63 (625 Alternately spliced γ 2 RGDs in Aα (95–97 2 Aα, 2 Bβ, 2 γ via 340,000 Thrombospondin
amino acids) chains and 572–574) disulfide bonds ?Collagen
Bβ = 56 (461 Phosphorylation of Aα α β – RGD Staphylococci
V 3
amino acids) 572–574 Factor XIII
γ = 47 (427 α β –C-terminal γ - Thrombin
IIb 3
amino acids) chain dodecamer
(400–411)
Vitronectin 1 chain = 75 Met → Thr RGD Same as subunits 75,000 and Glass
(458 amino polymorphism Somatomedin B 65,000+10,000 Plastic
acids) 2 Hemopexin Heparin
2 chain = Serine protease:
65 + 10 via serpin complexes
disulfide bonds PAI-1
uPAR
Factor XIII
Fibronectin 220 (2355 Types I, II, and III RGD (1493–1495) Heterodimer via 440,000 Fibrin
amino acids) repeats disulfide bonds Heparin
Alternately spliced Collagen
forms DNA
Staphylococci
Thrombo- 180 (1150 RGD (?functional) Trimer via 450,000 Calcium
spondin 1 amino acids) VTCG† disulfide bonds Plasminogen
α (I) Collagen Collagen
1
Epidermal growth Fibrinogen
factor Histidine-rich
Malaria antigen glycoprotein
Fibronectin
Laminin
Heparin
Osteopontin 32 (298 amino Phosphorylation RGD Hydroxyapatite
acids) Sulfation Plaque components
Laminin A = 400 YIGSR† A, B , B , via disul- 850,000 Collagen type IV
1
2
B = 215 (1765 RGD (?functional) fide bonds Nidogen/entactin
1
amino acids) EGF Osteonectin
B = 205 (1576 Heparin sulfate
2
amino acids) C1q
Plasminogen
Plasmin
Multimerin 155 or 167 kDa Large prepro-peptide RGD in N-terminal 450,000– Factor V
(1228 amino acids) region ~5,000,000
EGF
EGF, epidermal growth factor; PAI-1, plasminogen activator-1; RDG, arginine-glycine-aspartic acid sequence; uPAR, urine-type plasminogen
activator receptor; VWFA, VWFC, von Willebrand factor A and C repeats.

Kaushansky_chapter 112_p1829-1914.indd 1846 17/09/15 3:26 pm