Page 1970 - Williams Hematology ( PDFDrive )

1944 Part XII: Hemostasis and Thrombosis Chapter 113: Molecular Biology and Biochemistry of the Coagulation Factors 1945

176. Ogura M, Tanabe N, Nishioka J, et al: Biosynthesis and secretion of functional protein 208. Bouma B, Griffin JH: Human blood coagulation factor XI. Purification, properties, and
S by a human megakaryoblastic cell line (MEG-01). Blood 70:301–306, 1987. mechanism of activation by activated factor XII. J Biol Chem 252:6432, 1977.
177. Dahlbäck B: Protein S and C4b-binding protein: Components involved in the regula- 209. Naito K, Fujikawa K: Activation of human blood coagulation factor XI independent of
tion of the protein C anticoagulant system. Thromb Haemost 66:49–61, 1991. factor XII. Factor XI is activated by thrombin and factor XIa in the presence of nega-
178. Maillard C, Berruyer M, Serre CM, et al: Protein-S, a vitamin K-dependent protein, tively charged surfaces. J Biol Chem 266:7353–7358, 1991.
is a bone matrix component synthesized and secreted by osteoblasts. Endocrinology 210. Geng Y, Verhamme I, Smith S: The dimeric structure of factor XI and zymogen activa-
130:1599–1604, 1992. tion. Blood 121:3962–3970, 2013.
179. Walker FJ: Regulation of activated protein C by a new protein. A possible function for 211. Smith SB, Verhamme IM, Sun M-F, et al: Characterization of novel forms of coagula-
bovine protein S. J Biol Chem 255:5521–5524, 1980. tion factor XIa: Independence of factor XIa subunits in factor IX activation. J Biol Chem
180. van de Poel RH, Meijers JC, Bouma BN: C4b-binding protein inhibits the factor V-de- 283:6696–6705, 2008.
pendent but not the factor V-independent cofactor activity of protein S in the activated 212. Sun Y, Gailani D: Identification of a factor IX binding site on the third apple domain of
protein C-mediated inactivation of factor VIIIa. Thromb Haemost 85:761–765, 2001. activated factor XI. J Biol Chem 271:29023–29028, 1996.
181. Yegneswaran S, Wood GM, Esmon CT, Johnson AE: Protein S alters the active site 213. Sinha D, Marcinkiewicz M, Navaneetham D, Walsh PN: Macromolecular sub-
location of activated protein C above the membrane surface. A fluorescence resonance strate-binding exosites on both the heavy and light chains of factor XIa mediate the
energy transfer study of topography. J Biol Chem 272:25013–25021, 1997. formation of the Michaelis complex required for factor IX-activation. Biochemistry
182. Rosing J, Hoekema L, Nicolaes GA, et al: Effects of protein S and factor Xa on peptide 46:9830–9839, 2007.
bond cleavages during inactivation of factor Va and factor VaR506Q by activated pro- 214. Kravtsov DV, Matafonov A, Tucker EI, et al: Factor XI contributes to thrombin genera-
tein C. J Biol Chem 270:27852–27858, 1995. tion in the absence of factor XII. Blood 114:452, 2009.
183. Maurissen LF, Thomassen MC, Nicolaes GA, et al: Re-evaluation of the role of the pro- 215. Cheng Q, Tucker E, Pine M, et al: A role for factor XIIa–mediated factor XI activation
tein S-C4b binding protein complex in activated protein C-catalyzed factor Va-inacti- in thrombus formation in vivo. Blood 116:3981–3990, 2010.
vation. Blood 111:3034–3041, 2008. 216. He R, Chen D, He S: Factor XI: Hemostasis, thrombosis, and antithrombosis. Thromb
184. Dahlback B: The tale of protein S and C4b-binding protein, a story of affection. Thromb Res 129:541–550, 2012.
Haemost 98:90–96, 2007. 217. von dem Borne PA, Cox LM, Bouma BN: Factor XI enhances fibrin generation and
185. Reglińska-Matveyev N, Andersson H, Rezende S, et al: TFPI cofactor function of pro- inhibits fibrinolysis in a coagulation model initiated by surface-coated tissue factor.
tein S: Essential role of the protein S SHBG-like domain. Blood 123:3979–3988, 2014. Blood Coagul Fibrinolysis 17:251–257, 2006.
186. Suleiman L, Négrier C, Boukerche H: Protein S: A multifunctional anticoagulant vita- 218. von dem Borne PA, Meijers JC, Bouma BN: Feedback activation of factor XI by
min K-dependent protein at the crossroads of coagulation, inflammation, angiogenesis, thrombin in plasma results in additional formation of thrombin that protects fibrin
and cancer. Crit Rev Oncol Hematol 88:637–654, 2013. clots from fibrinolysis. Blood 86:3035–3042, 1995.
187. van der Meer JH, van der Poll T, van ‘t Veer C: TAM receptors, Gas6, and protein S: 219. Baglia FA, Gailani D, López JA, Walsh PN: Identification of a binding site for glyco-
Roles in inflammation and hemostasis. Blood 123:2460–2470, 2014. protein Ibalpha in the Apple 3 domain of factor XI. J Biol Chem 279:45470–45476,
188. Ploos van Amstel HK, Reitsma PH, van der Logt CP, Bertina RM: Intron-exon organi- 2004.
zation of the active human protein S gene PS alpha and its pseudogene PS beta: Dupli- 220. White-Adams TC, Berny MA, Tucker EI, et al: Identification of coagulation factor XI
cation and silencing during primate evolution. Biochemistry 29:7853–7861, 1990. as a ligand for platelet apolipoprotein E receptor 2 (ApoER2). Arterioscler Thromb Vasc
189. Gómez E, Ledford MR, Pegelow CH, et al: Homozygous protein S deficiency due to Biol 29:1602–1607, 2009.
a one base pair deletion that leads to a stop codon in exon III of the protein S gene. 221. Gailani D, Ho D, Sun MF, et al: Model for a factor IX activation complex on blood
Thromb Haemost 71:723–726, 1994. platelets: Dimeric conformation of factor XIa is essential. Blood 97:3117–3122, 2001.
190. Marlar RA, Gausman JN: Protein S abnormalities: A diagnostic nightmare. Am J Hema- 222. Knauer DJ, Majumdar D, Fong PC, Knauer MF: SERPIN regulation of factor XIa. The
tol 86:418–421, 2011. novel observation that protease nexin 1 in the presence of heparin is a more potent
191. Pintao MC, Garcia AA, Borgel D, et al: Gross deletions/duplications in PROS1 are rela- inhibitor of factor XIa than C1 inhibitor. J Biol Chem 275:37340–37346, 2000.
tively common in point mutation-negative hereditary protein S deficiency. Hum Genet 223. Cronlund AL, Walsh PN: A low molecular weight platelet inhibitor of factor XIa: Purifi-
126:449–456, 2009. cation, characterization, and possible role in blood coagulation. Biochemistry 31:1685–
192. Pintao MC, Ribeiro DD, Bezemer ID, et al: Protein S levels and the risk of venous 1694, 1992.
thrombosis: Results from the MEGA case-control study. Blood 122:3210–3219, 2013. 224. Ragni MV, Sinha D, Seaman F, et al: Comparison of bleeding tendency, factor XI coagu-
193. Giblin JP, Hewlett LJ, Hannah MJ: Basal secretion of von Willebrand factor from lant activity, and factor XI antigen in 25 factor XI-deficient kindreds. Blood 65:719–724,
human endothelial cells. Blood 112:957–964, 2008. 1985.
194. van Schooten CJ, Shahbazi S, Groot E, et al: Macrophages contribute to the cellular 225. Asakai R, Chung DW, Davie EW, Seligsohn U: Factor XI deficiency in Ashkenazi Jews
uptake of von Willebrand factor and factor VIII in vivo. Blood 112:1704–1712, 2008. in Israel. N Engl J Med 325:153–158, 1991.
195. Furlan M, Robles R, Lammle B: Partial purification and characterization of a protease 226. Meijers JC, Tekelenburg WL, Bouma BN, et al: High levels of coagulation factor XI as a
from human plasma cleaving von Willebrand factor to fragments produced by in vivo risk factor for venous thrombosis. N Engl J Med 342:696–701, 2000.
proteolysis. Blood 87:4223–4234, 1996. 227. Bezemer ID, Bare LA, Doggen CJ, et al: Gene variants associated with deep vein throm-
196. Bonthron DT, Handin RI, Kaufman RJ, et al: Structure of pre-pro-von Willebrand fac- bosis. JAMA 299:1306–1314, 2008.
tor and its expression in heterologous cells. Nature 324:270–273, 1986. 228. Maas C, Oschatz C, Renne T: The plasma contact system 2.0. Semin Thromb Hemost
197. Huizinga EG, Tsuji S, Romijn RA, et al: Structures of glycoprotein Ibalpha and its com- 37:375–381, 2011.
plex with von Willebrand factor A1 domain. Science 297:1176–1179, 2002. 229. Ratnoff O, Colopy J: A familial hemorrhagic trait associated with a deficiency of a
198. Foster PA, Fulcher CA, Marti T, et al: A major factor VIII binding domain resides clot-promoting fraction of plasma. J Clin Invest 34:602–613, 1955.
within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol 230. McMullen BA, Fujikawa K: Amino acid sequence of the heavy chain of human alpha-
Chem 262:8443–8446, 1987. factor XIIa (activated Hageman factor). J Biol Chem 260:5328–5341, 1985.
199. Mancuso DJ, Tuley EA, Westfield LA, et al: Human von Willebrand factor gene and 231. Harris RJ, Ling VT, Spellman W: O-linked fucose is present in the first epidermal
pseudogene: Structural analysis and differentiation by polymerase chain reaction. Bio- growth factor domain of factor XI1 but not protein C. J Biol Chem 15:5102–5107,
chemistry 30:253–269, 1991. 1992.
200. Smith NL, Chen MH, Dehghan A, et al: Novel associations of multiple genetic loci 232. Cochrane CG, Revak SD, Wuepper KD: Activation of Hageman factor in solid and fluid
with plasma levels of factor VII, factor VIII, and von Willebrand factor: The CHARGE phases. A critical role of kallikrein. J Exp Med 138:1564–1583, 1973.
(Cohorts for Heart and Aging Research in Genome Epidemiology) Consortium. Circu- 233. Samuel M, Pixley RA, Villanueva MA, et al: Human factor XII (Hageman factor) auto-
lation 121:1382–1392, 2010. activation by dextran sulfate. Circular dichroism, fluorescence, and ultraviolet differ-
201. Smith NL, Rice KM, Bovill EG, et al: Genetic variation associated with plasma von ence spectroscopic studies. J Biol Chem 267:19691–19697, 1992.
Willebrand factor levels and the risk of incident venous thrombosis. Blood 117:6007– 234. Engel R, Brain CM, Paget J, et al: Single-chain factor XII exhibits activity when com-
6011, 2011. plexed to polyphosphate. J Thromb Haemost 12:1513–1522, 2014.
202. Aggeler PM, White SG, Glendening MB, et al: Plasma thromboplastin component 235. Ratnoff OD, Saito H: Amidolytic properties of single-chain activated Hageman factor.
(PTC) deficiency; a new disease resembling hemophilia. Proc Soc Exp Biol Med 79:692– Proc Natl Acad Sci U S A 76:1461–1463, 1979.
694, 1952. 236. Griffin JH: Role of surface in surface-dependent activation of Hageman factor (blood
203. Rosenthal RL, Dreskin OH, Rosenthal N: New hemophilia-like disease caused by defi- coagulation factor XII). Proc Natl Acad Sci U S A 75:1998–2002, 1978.
ciency of a third plasma thromboplastin factor. Proc Soc Exp Biol Med 82:171–174, 1953. 237. Clarke BJ, Côté HC, Cool DE, et al: Mapping of a putative surface-binding site of
204. Thompson RE, Mandle R, Kaplan AP: Association of factor XI and high molecular human coagulation factor XII. J Biol Chem 264:11497–11502, 1989.
weight kininogen in human plasma. J Clin Invest 60:1376–1380, 1977. 238. Citarella F, Ravon DM, Pascucci B, et al: Structure/function analysis of human factor
205. Kurachi K, Fujikawa K, Davie EW: Mechanism of activation of bovine factor XI by XII using recombinant deletion mutants. Evidence for an additional region involved in
factor XII and factor XIIa. Biochemistry 19:1330–1338, 1980. the binding to negatively charged surfaces. Eur J Biochem 238:240–249, 1996.
206. Emsley J, McEwan PA, Gailani D: Structure and function of factor XI. Blood 115:2569– 239. Citarella F, te Velthuis H, Helmer-Citterich M, Hack CE: Identification of a putative
2577, 2010. binding site for negatively charged surfaces in the fibronectin type II domain of human
207. Papagrigoriou E, McEwan PA, Walsh PN, Emsley J: Crystal structure of the factor XI factor XII—An immunochemical and homology modeling approach. Thromb Haemost
zymogen reveals a pathway for transactivation. Nat Struct Mol Biol 13:557–558, 2006. 84:1057–1065, 2000.

Kaushansky_chapter 113_p1915-1948.indd 1945 9/21/15 2:41 PM