메뉴 건너뛰기




Volumn 29, Issue 3, 2015, Pages 509-524

Ultralarge Von Willebrand Factor-Induced Platelet Clumping and Activation of the Alternative Complement Pathway in Thrombotic Thrombocytopenic Purpura and the Hemolytic-Uremic Syndromes

Author keywords

Alternative complement pathway; Factor H; Thrombotic microangiopathies; VWF

Indexed keywords

ALTERNATIVE COMPLEMENT PATHWAY C3 C5 CONVERTASE; CD59 ANTIGEN; COMPLEMENT COMPONENT C3; COMPLEMENT COMPONENT C3B; COMPLEMENT COMPONENT C5; COMPLEMENT COMPONENT C5B; COMPLEMENT COMPONENT C9; COMPLEMENT FACTOR H; COMPLEMENT FACTOR I; COMPLEMENT MEMBRANE ATTACK COMPLEX; GLYCOSYLPHOSPHATIDYLINOSITOL ANCHORED PROTEIN; MEMBRANE COFACTOR PROTEIN; THROMBOMODULIN; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

EID: 84930043360     PISSN: 08898588     EISSN: 15581977     Source Type: Journal    
DOI: 10.1016/j.hoc.2015.01.008     Document Type: Review
Times cited : (24)

References (68)
  • 1
    • 0037158606 scopus 로고    scopus 로고
    • Thrombotic microangiopathies
    • Moake J.L. Thrombotic microangiopathies. NEngl J Med 2002, 347(8):589-600.
    • (2002) NEngl J Med , vol.347 , Issue.8 , pp. 589-600
    • Moake, J.L.1
  • 2
    • 84875597120 scopus 로고    scopus 로고
    • Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis
    • Turner N.A., Moake J. Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis. PLoS One 2013, 8(3):e59372.
    • (2013) PLoS One , vol.8 , Issue.3 , pp. e59372
    • Turner, N.A.1    Moake, J.2
  • 3
    • 0032569884 scopus 로고    scopus 로고
    • Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome
    • Furlan M., Robles R., Galbusera M., et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. NEngl J Med 1998, 339(22):1578-1584.
    • (1998) NEngl J Med , vol.339 , Issue.22 , pp. 1578-1584
    • Furlan, M.1    Robles, R.2    Galbusera, M.3
  • 4
    • 0030973267 scopus 로고    scopus 로고
    • Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura
    • Furlan M., Robles R., Solenthaler M., et al. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 1997, 89(9):3097-3103.
    • (1997) Blood , vol.89 , Issue.9 , pp. 3097-3103
    • Furlan, M.1    Robles, R.2    Solenthaler, M.3
  • 5
    • 0032569840 scopus 로고    scopus 로고
    • Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura
    • Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. NEngl J Med 1998, 339(22):1585-1594.
    • (1998) NEngl J Med , vol.339 , Issue.22 , pp. 1585-1594
    • Tsai, H.M.1    Lian, E.C.2
  • 6
    • 0041411081 scopus 로고    scopus 로고
    • Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression
    • Garg A.X., Suri R.S., Barrowman N., et al. Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression. JAMA 2003, 290:1360-1370.
    • (2003) JAMA , vol.290 , pp. 1360-1370
    • Garg, A.X.1    Suri, R.S.2    Barrowman, N.3
  • 7
    • 84867911677 scopus 로고    scopus 로고
    • Shigatoxin-associated hemolytic uremic syndrome: current molecular mechanisms and future therapies
    • Keir L.S., Marks S.D., Kim J.J. Shigatoxin-associated hemolytic uremic syndrome: current molecular mechanisms and future therapies. Drug Des Devel Ther 2012, 6:195-208.
    • (2012) Drug Des Devel Ther , vol.6 , pp. 195-208
    • Keir, L.S.1    Marks, S.D.2    Kim, J.J.3
  • 8
    • 28844503491 scopus 로고    scopus 로고
    • Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers
    • Nolasco L.H., Turner N.A., Bernardo A., et al. Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers. Blood 2005, 106(13):4199-4209.
    • (2005) Blood , vol.106 , Issue.13 , pp. 4199-4209
    • Nolasco, L.H.1    Turner, N.A.2    Bernardo, A.3
  • 9
    • 84895750286 scopus 로고    scopus 로고
    • Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab
    • Delmas Y., Vendrely B., Clouzeau B., et al. Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab. Nephrol Dial Transplant 2014, 29(3):565-572.
    • (2014) Nephrol Dial Transplant , vol.29 , Issue.3 , pp. 565-572
    • Delmas, Y.1    Vendrely, B.2    Clouzeau, B.3
  • 10
    • 84868562798 scopus 로고    scopus 로고
    • Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry
    • Kielstein J.T., Beutel G., Fleig S., et al. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant 2012, 27(10):3807-3815.
    • (2012) Nephrol Dial Transplant , vol.27 , Issue.10 , pp. 3807-3815
    • Kielstein, J.T.1    Beutel, G.2    Fleig, S.3
  • 11
    • 0023946001 scopus 로고
    • Cytotoxin producing Escherichia coli and the hemolytic uremic syndrome
    • Cleary T.G. Cytotoxin producing Escherichia coli and the hemolytic uremic syndrome. Pediatr Clin North Am 1988, 35:485-501.
    • (1988) Pediatr Clin North Am , vol.35 , pp. 485-501
    • Cleary, T.G.1
  • 12
    • 0042237838 scopus 로고    scopus 로고
    • Molecular and functional analysis of Shiga toxin-induced response patterns in human vascular endothelial cells
    • Matussek A., Lauber J., Bergau A., et al. Molecular and functional analysis of Shiga toxin-induced response patterns in human vascular endothelial cells. Blood 2003, 102(4):1323-1332.
    • (2003) Blood , vol.102 , Issue.4 , pp. 1323-1332
    • Matussek, A.1    Lauber, J.2    Bergau, A.3
  • 13
    • 84864372320 scopus 로고    scopus 로고
    • Asingle-step purification and molecular characterization of functional Shiga toxin 2 variants from pathogenic Escherichia coli
    • He X., Quinones B., McMahon S., et al. Asingle-step purification and molecular characterization of functional Shiga toxin 2 variants from pathogenic Escherichia coli. Toxins (Basel) 2012, 4(7):487-504.
    • (2012) Toxins (Basel) , vol.4 , Issue.7 , pp. 487-504
    • He, X.1    Quinones, B.2    McMahon, S.3
  • 14
    • 79952078677 scopus 로고    scopus 로고
    • AB toxins: a paradigm switch from deadly to desirable
    • Odumosu O., Nicholas D., Yano H., et al. AB toxins: a paradigm switch from deadly to desirable. Toxins (Basel) 2010, 2:1612-1645.
    • (2010) Toxins (Basel) , vol.2 , pp. 1612-1645
    • Odumosu, O.1    Nicholas, D.2    Yano, H.3
  • 15
    • 15244348050 scopus 로고    scopus 로고
    • Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome
    • Tarr P.I., Gordon C.A., Chandler W.L. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 2005, 365(9464):1073-1086.
    • (2005) Lancet , vol.365 , Issue.9464 , pp. 1073-1086
    • Tarr, P.I.1    Gordon, C.A.2    Chandler, W.L.3
  • 16
    • 84867993202 scopus 로고    scopus 로고
    • Renal and neurological involvement in typical Shiga toxin-associated HUS
    • Trachtman H., Austin C., Lewinski M., et al. Renal and neurological involvement in typical Shiga toxin-associated HUS. Nat Rev Nephrol 2012, 8(11):658-669.
    • (2012) Nat Rev Nephrol , vol.8 , Issue.11 , pp. 658-669
    • Trachtman, H.1    Austin, C.2    Lewinski, M.3
  • 18
    • 77952682366 scopus 로고    scopus 로고
    • Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome
    • Maga T.K., Nishimura C.J., Weaver A.E., et al. Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome. Hum Mutat 2010, 31(6):E1445-E1460.
    • (2010) Hum Mutat , vol.31 , Issue.6 , pp. E1445-E1460
    • Maga, T.K.1    Nishimura, C.J.2    Weaver, A.E.3
  • 19
    • 34047200899 scopus 로고    scopus 로고
    • Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome
    • Zipfel P.F., Edey M., Heinen S., et al. Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome. PLoS Genet 2007, 3(3):e41.
    • (2007) PLoS Genet , vol.3 , Issue.3 , pp. e41
    • Zipfel, P.F.1    Edey, M.2    Heinen, S.3
  • 20
    • 54049137505 scopus 로고    scopus 로고
    • Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome
    • Frémeaux-Bacchi V., Miller E.C., Liszewski M.K., et al. Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood 2008, 112(13):4948-4952.
    • (2008) Blood , vol.112 , Issue.13 , pp. 4948-4952
    • Frémeaux-Bacchi, V.1    Miller, E.C.2    Liszewski, M.K.3
  • 21
    • 33846094404 scopus 로고    scopus 로고
    • Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome
    • Goicoechea de Jorge E., Harris C.L., Esparza-Gordillo J., et al. Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc Natl Acad Sci USA 2007, 104(1):240-245.
    • (2007) Proc Natl Acad Sci USA , vol.104 , Issue.1 , pp. 240-245
    • Goicoechea de Jorge, E.1    Harris, C.L.2    Esparza-Gordillo, J.3
  • 22
    • 79551595553 scopus 로고    scopus 로고
    • Alternative complement pathway assessment in patients with atypical HUS
    • Roumenina L.T., Loirat C., Dragon-Durey M.A., et al. Alternative complement pathway assessment in patients with atypical HUS. JImmunol Methods 2011, 365(1-2):8-26.
    • (2011) JImmunol Methods , vol.365 , Issue.1-2 , pp. 8-26
    • Roumenina, L.T.1    Loirat, C.2    Dragon-Durey, M.A.3
  • 23
    • 0344136374 scopus 로고
    • Interaction between the third complement protein and cell surface macromolecules
    • Law S.K., Levine R.P. Interaction between the third complement protein and cell surface macromolecules. Proc Natl Acad Sci USA 1977, 74(7):2701-2705.
    • (1977) Proc Natl Acad Sci USA , vol.74 , Issue.7 , pp. 2701-2705
    • Law, S.K.1    Levine, R.P.2
  • 24
    • 57649162572 scopus 로고    scopus 로고
    • Discrimination between host and pathogens by the complement system
    • Pangburn M.K., Ferreira V.P., Cortes C. Discrimination between host and pathogens by the complement system. Vaccine 2008, 26(Suppl 8):I15-I21.
    • (2008) Vaccine , vol.26 , pp. I15-I21
    • Pangburn, M.K.1    Ferreira, V.P.2    Cortes, C.3
  • 25
    • 0031043135 scopus 로고    scopus 로고
    • The internal thioester and the covalent binding properties of the complement proteins C3 and C4
    • Law S.K., Dodds A.W. The internal thioester and the covalent binding properties of the complement proteins C3 and C4. Protein Sci 1997, 6(2):263-274.
    • (1997) Protein Sci , vol.6 , Issue.2 , pp. 263-274
    • Law, S.K.1    Dodds, A.W.2
  • 26
    • 0018151413 scopus 로고
    • Initiation of the alternative pathway of complement: recognition of activators by bound C3b and assembly of the entire pathway from six isolated proteins
    • Schreiber R.D., Pangburn M.K., Lesavre P.H., et al. Initiation of the alternative pathway of complement: recognition of activators by bound C3b and assembly of the entire pathway from six isolated proteins. Proc Natl Acad Sci USA 1978, 75(8):3948-3952.
    • (1978) Proc Natl Acad Sci USA , vol.75 , Issue.8 , pp. 3948-3952
    • Schreiber, R.D.1    Pangburn, M.K.2    Lesavre, P.H.3
  • 27
    • 0015744783 scopus 로고
    • Formation of a hemolytically active cellular intermediate by the interaction between properdin, factors B and D and the activated third component of complement
    • Fearon D.T., Austen K.F., Ruddy S. Formation of a hemolytically active cellular intermediate by the interaction between properdin, factors B and D and the activated third component of complement. JExp Med 1973, 138(6):1305-1313.
    • (1973) JExp Med , vol.138 , Issue.6 , pp. 1305-1313
    • Fearon, D.T.1    Austen, K.F.2    Ruddy, S.3
  • 28
    • 0000719411 scopus 로고
    • The properdin system and immunity. I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena
    • Pillemer L., Blum L., Lepow I.H., et al. The properdin system and immunity. I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena. Science 1954, 120(3112):279-285.
    • (1954) Science , vol.120 , Issue.3112 , pp. 279-285
    • Pillemer, L.1    Blum, L.2    Lepow, I.H.3
  • 29
    • 0012042656 scopus 로고
    • Control of the amplification convertase of complement by the plasma protein beta1H
    • Weiler J.M., Daha M.R., Austen K.F., et al. Control of the amplification convertase of complement by the plasma protein beta1H. Proc Natl Acad Sci USA 1976, 73(9):3268-3272.
    • (1976) Proc Natl Acad Sci USA , vol.73 , Issue.9 , pp. 3268-3272
    • Weiler, J.M.1    Daha, M.R.2    Austen, K.F.3
  • 30
    • 0024159572 scopus 로고
    • C5 convertase of the alternative complement pathway: covalent linkage between two C3b molecules within the trimolecular complex enzyme
    • Kinoshita T., Takata Y., Kozono H., et al. C5 convertase of the alternative complement pathway: covalent linkage between two C3b molecules within the trimolecular complex enzyme. JImmunol 1988, 141(11):3895.
    • (1988) JImmunol , vol.141 , Issue.11 , pp. 3895
    • Kinoshita, T.1    Takata, Y.2    Kozono, H.3
  • 31
    • 0035865060 scopus 로고    scopus 로고
    • Formation of high-affinity C5 convertases of the alternative pathway of complement
    • Rawal N., Pangburn M. Formation of high-affinity C5 convertases of the alternative pathway of complement. JImmunol 2001, 166(4):2635-2642.
    • (2001) JImmunol , vol.166 , Issue.4 , pp. 2635-2642
    • Rawal, N.1    Pangburn, M.2
  • 32
    • 3042839778 scopus 로고    scopus 로고
    • Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow
    • Bernardo A., Ball C., Nolasco L., et al. Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow. Blood 2004, 104(1):100-106.
    • (2004) Blood , vol.104 , Issue.1 , pp. 100-106
    • Bernardo, A.1    Ball, C.2    Nolasco, L.3
  • 33
    • 78149336370 scopus 로고    scopus 로고
    • Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice
    • Huang J., Motto D.G., Bundle D.R., et al. Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice. Blood 2010, 116(18):3653-3659.
    • (2010) Blood , vol.116 , Issue.18 , pp. 3653-3659
    • Huang, J.1    Motto, D.G.2    Bundle, D.R.3
  • 34
    • 0034648102 scopus 로고    scopus 로고
    • Expression of complement messenger RNAs by human endothelial cells
    • Klegeris A., Bissonnette C.J., Dorovini-Zis K., et al. Expression of complement messenger RNAs by human endothelial cells. Brain Res 2000, 871(1):1-6.
    • (2000) Brain Res , vol.871 , Issue.1 , pp. 1-6
    • Klegeris, A.1    Bissonnette, C.J.2    Dorovini-Zis, K.3
  • 35
    • 0035076705 scopus 로고    scopus 로고
    • Detection of mRNA for the terminal complement components C5, C6, C8 and C9 in human umbilical vein endothelial cells invitro
    • Langeggen H., Berge K.E., Macor P., et al. Detection of mRNA for the terminal complement components C5, C6, C8 and C9 in human umbilical vein endothelial cells invitro. APMIS 2001, 109(1):73-78.
    • (2001) APMIS , vol.109 , Issue.1 , pp. 73-78
    • Langeggen, H.1    Berge, K.E.2    Macor, P.3
  • 36
    • 0033945557 scopus 로고    scopus 로고
    • The endothelium is an extrahepatic site of synthesis of the seventh component of the complement system
    • Langeggen H., Pausa M., Johnson E., et al. The endothelium is an extrahepatic site of synthesis of the seventh component of the complement system. Clin Exp Immunol 2000, 121(1):69-76.
    • (2000) Clin Exp Immunol , vol.121 , Issue.1 , pp. 69-76
    • Langeggen, H.1    Pausa, M.2    Johnson, E.3
  • 37
    • 0028350821 scopus 로고
    • Complement component C7. Assessment of invivo synthesis after liver transplantation reveals that hepatocytes do not synthesize the majority of human C7
    • Wurzner R., Joysey V.C., Lachmann P.J. Complement component C7. Assessment of invivo synthesis after liver transplantation reveals that hepatocytes do not synthesize the majority of human C7. JImmunol 1994, 152(9):4624-4629.
    • (1994) JImmunol , vol.152 , Issue.9 , pp. 4624-4629
    • Wurzner, R.1    Joysey, V.C.2    Lachmann, P.J.3
  • 38
    • 0032525207 scopus 로고    scopus 로고
    • Endothelial cells in physiology and in the pathophysiology of vascular disorders
    • Cines D.B., Pollak E.S., Buck C.A., et al. Endothelial cells in physiology and in the pathophysiology of vascular disorders. Blood 1998, 91(10):3527-3561.
    • (1998) Blood , vol.91 , Issue.10 , pp. 3527-3561
    • Cines, D.B.1    Pollak, E.S.2    Buck, C.A.3
  • 39
    • 84902589905 scopus 로고    scopus 로고
    • Biomarkers of the alternative pathway and terminal complement activity at presentation confirms the clinical diagnosis of aHUS and differentiates aHUS from TTP
    • Cataland S.R., Holers V.M., Geyer S., et al. Biomarkers of the alternative pathway and terminal complement activity at presentation confirms the clinical diagnosis of aHUS and differentiates aHUS from TTP. Blood 2014, 123:3733-3738.
    • (2014) Blood , vol.123 , pp. 3733-3738
    • Cataland, S.R.1    Holers, V.M.2    Geyer, S.3
  • 40
    • 73649122762 scopus 로고    scopus 로고
    • Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome
    • Thurman J.M., Marians R., Emlen W., et al. Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 2009, 4(12):1920-1924.
    • (2009) Clin J Am Soc Nephrol , vol.4 , Issue.12 , pp. 1920-1924
    • Thurman, J.M.1    Marians, R.2    Emlen, W.3
  • 41
    • 0024386370 scopus 로고
    • Complement proteins C5b-9 induce secretion of high molecular weight multimers of endothelial von Willebrand factor and translocation of granule membrane protein GMP-140 to the cell surface
    • Hattori R., Hamilton K.K., McEver R.P., et al. Complement proteins C5b-9 induce secretion of high molecular weight multimers of endothelial von Willebrand factor and translocation of granule membrane protein GMP-140 to the cell surface. JBiol Chem 1989, 264(15):9053-9060.
    • (1989) JBiol Chem , vol.264 , Issue.15 , pp. 9053-9060
    • Hattori, R.1    Hamilton, K.K.2    McEver, R.P.3
  • 42
    • 84860474599 scopus 로고    scopus 로고
    • Complement activation in thrombotic thrombocytopenic purpura
    • Reti M., Farkas P., Csuka D., et al. Complement activation in thrombotic thrombocytopenic purpura. JThromb Haemost 2012, 10(5):791-798.
    • (2012) JThromb Haemost , vol.10 , Issue.5 , pp. 791-798
    • Reti, M.1    Farkas, P.2    Csuka, D.3
  • 43
    • 20144365269 scopus 로고    scopus 로고
    • Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies
    • Ruiz-Torres M.P., Casiraghi F., Galbusera M., et al. Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies. Thromb Haemost 2005, 93(3):443-452.
    • (2005) Thromb Haemost , vol.93 , Issue.3 , pp. 443-452
    • Ruiz-Torres, M.P.1    Casiraghi, F.2    Galbusera, M.3
  • 44
    • 84861526957 scopus 로고    scopus 로고
    • Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura
    • Chapin J., Weksler B., Magro C., et al. Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. Br J Haematol 2012, 157(6):772-774.
    • (2012) Br J Haematol , vol.157 , Issue.6 , pp. 772-774
    • Chapin, J.1    Weksler, B.2    Magro, C.3
  • 45
    • 84880700770 scopus 로고    scopus 로고
    • Use of eculizumab in the treatment of a case of refractory, ADAMTS13-deficient thrombotic thrombocytopenic purpura: additional data and clinical follow-up
    • Tsai E., Chapin J., Laurence J.C., et al. Use of eculizumab in the treatment of a case of refractory, ADAMTS13-deficient thrombotic thrombocytopenic purpura: additional data and clinical follow-up. Br J Haematol 2013, 162(4):558-559.
    • (2013) Br J Haematol , vol.162 , Issue.4 , pp. 558-559
    • Tsai, E.1    Chapin, J.2    Laurence, J.C.3
  • 46
    • 0014177784 scopus 로고
    • Nonlethal hemolytic and uremic syndromes in children: an electron-microscope study of renal biopsies from six cases
    • Courteciosse V., Habib R., Monnier C. Nonlethal hemolytic and uremic syndromes in children: an electron-microscope study of renal biopsies from six cases. Exp Mol Pathol 1967, 7:327-347.
    • (1967) Exp Mol Pathol , vol.7 , pp. 327-347
    • Courteciosse, V.1    Habib, R.2    Monnier, C.3
  • 47
    • 0015848888 scopus 로고
    • Platelet, erythrocyte, and fibrinogen kinetics in the hemolytic-uremic syndrome of infancy
    • Katz J., Krawitz S., Sacks P.V., et al. Platelet, erythrocyte, and fibrinogen kinetics in the hemolytic-uremic syndrome of infancy. JPediatr 1973, 83(5):739-748.
    • (1973) JPediatr , vol.83 , Issue.5 , pp. 739-748
    • Katz, J.1    Krawitz, S.2    Sacks, P.V.3
  • 48
    • 0034926743 scopus 로고    scopus 로고
    • Response to single and divided doses of Shiga toxin-1 in a primate model of hemolytic uremic syndrome
    • Siegler R.L., Pysher T.J., Tesh V.L., et al. Response to single and divided doses of Shiga toxin-1 in a primate model of hemolytic uremic syndrome. JAm Soc Nephrol 2001, 12:1458-1467.
    • (2001) JAm Soc Nephrol , vol.12 , pp. 1458-1467
    • Siegler, R.L.1    Pysher, T.J.2    Tesh, V.L.3
  • 49
    • 0036760183 scopus 로고    scopus 로고
    • Von Willebrand factor expression in a Shiga toxin-mediated primate model of hemolytic uremic syndrome
    • Pysher T.J., Siegler R.L., Tesh V.L., et al. von Willebrand factor expression in a Shiga toxin-mediated primate model of hemolytic uremic syndrome. Pediatr Dev Pathol 2002, 5(5):472-479.
    • (2002) Pediatr Dev Pathol , vol.5 , Issue.5 , pp. 472-479
    • Pysher, T.J.1    Siegler, R.L.2    Tesh, V.L.3
  • 50
    • 0043203072 scopus 로고    scopus 로고
    • Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods
    • Studt J.D., Bohm M., Budde U., et al. Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. JThromb Haemost 2003, 1(9):1882-1887.
    • (2003) JThromb Haemost , vol.1 , Issue.9 , pp. 1882-1887
    • Studt, J.D.1    Bohm, M.2    Budde, U.3
  • 51
    • 0035035397 scopus 로고    scopus 로고
    • Von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli 0157: H7-associated hemolytic uremic syndrome
    • Tsai H.M., Chandler W.L., Sarode R., et al. von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli 0157: H7-associated hemolytic uremic syndrome. Pediatr Res 2001, 49:653-659.
    • (2001) Pediatr Res , vol.49 , pp. 653-659
    • Tsai, H.M.1    Chandler, W.L.2    Sarode, R.3
  • 52
    • 84887840553 scopus 로고    scopus 로고
    • Interaction of Shiga toxin with the A-domains and multimers of von Willebrand Factor
    • Lo N.C., Turner N.A., Cruz M.A., et al. Interaction of Shiga toxin with the A-domains and multimers of von Willebrand Factor. JBiol Chem 2013, 288(46):33118-33123.
    • (2013) JBiol Chem , vol.288 , Issue.46 , pp. 33118-33123
    • Lo, N.C.1    Turner, N.A.2    Cruz, M.A.3
  • 53
    • 79960435714 scopus 로고    scopus 로고
    • Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis
    • Morigi M., Galbusera M., Gastoldi S., et al. Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis. JImmunol 2011, 187(1):172-180.
    • (2011) JImmunol , vol.187 , Issue.1 , pp. 172-180
    • Morigi, M.1    Galbusera, M.2    Gastoldi, S.3
  • 54
    • 70350693665 scopus 로고    scopus 로고
    • Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome
    • Orth D., Khan A.B., Naim A., et al. Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome. JImmunol 2009, 182(10):6394-6400.
    • (2009) JImmunol , vol.182 , Issue.10 , pp. 6394-6400
    • Orth, D.1    Khan, A.B.2    Naim, A.3
  • 55
    • 84880648308 scopus 로고    scopus 로고
    • Shiga toxin 2 reduces complement inhibitor CD59 expression on human renal tubular epithelial and glomerular endothelial cells
    • Ehrlenbach S., Rosales A., Posch W., et al. Shiga toxin 2 reduces complement inhibitor CD59 expression on human renal tubular epithelial and glomerular endothelial cells. Infect Immun 2013, 81(8):2678-2685.
    • (2013) Infect Immun , vol.81 , Issue.8 , pp. 2678-2685
    • Ehrlenbach, S.1    Rosales, A.2    Posch, W.3
  • 56
    • 84887122022 scopus 로고    scopus 로고
    • Human complement factor H is a reductase for large soluble von Willebrand factor multimers-brief report
    • Nolasco L., Nolasco J., Feng S., et al. Human complement factor H is a reductase for large soluble von Willebrand factor multimers-brief report. Arterioscler Thromb Vasc Biol 2013, 33(11):2524-2528.
    • (2013) Arterioscler Thromb Vasc Biol , vol.33 , Issue.11 , pp. 2524-2528
    • Nolasco, L.1    Nolasco, J.2    Feng, S.3
  • 57
    • 0017595341 scopus 로고
    • Breakdown products of C3 and factor B in hemolytic-uremic syndrome
    • Kim Y., Miller K., Michael A.F. Breakdown products of C3 and factor B in hemolytic-uremic syndrome. JLab Clin Med 1977, 89(4):845-850.
    • (1977) JLab Clin Med , vol.89 , Issue.4 , pp. 845-850
    • Kim, Y.1    Miller, K.2    Michael, A.F.3
  • 58
    • 0018851755 scopus 로고
    • The complement system in hemolytic-uremic syndrome in childhood
    • Monnens L., Molenaar J., Lambert P.H., et al. The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 1980, 13(4):168-171.
    • (1980) Clin Nephrol , vol.13 , Issue.4 , pp. 168-171
    • Monnens, L.1    Molenaar, J.2    Lambert, P.H.3
  • 59
    • 0026666338 scopus 로고
    • Hypocomplementemia and leukocytosis in diarrhea-associated hemolytic uremic syndrome
    • Robson W.L., Leung A.K., Fick G.H., et al. Hypocomplementemia and leukocytosis in diarrhea-associated hemolytic uremic syndrome. Nephron 1992, 62(3):296-299.
    • (1992) Nephron , vol.62 , Issue.3 , pp. 296-299
    • Robson, W.L.1    Leung, A.K.2    Fick, G.H.3
  • 60
    • 79959776422 scopus 로고    scopus 로고
    • Eculizumab in severe Shiga-toxin-associated HUS
    • Lapeyraque A.L., Malina M., Fremeaux-Bacchi V., et al. Eculizumab in severe Shiga-toxin-associated HUS. NEngl J Med 2011, 364(26):2561-2563.
    • (2011) NEngl J Med , vol.364 , Issue.26 , pp. 2561-2563
    • Lapeyraque, A.L.1    Malina, M.2    Fremeaux-Bacchi, V.3
  • 61
    • 84886851977 scopus 로고    scopus 로고
    • Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome
    • Feng S., Eyler S.J., Zhang Y., et al. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome. Blood 2013, 122:1487-1493.
    • (2013) Blood , vol.122 , pp. 1487-1493
    • Feng, S.1    Eyler, S.J.2    Zhang, Y.3
  • 62
    • 84872511153 scopus 로고    scopus 로고
    • Complement activation in thrombotic microangiopathies
    • Feng S., Kroll M.H., Nolasco L., et al. Complement activation in thrombotic microangiopathies. Br J Haematol 2013, 160(3):404-406.
    • (2013) Br J Haematol , vol.160 , Issue.3 , pp. 404-406
    • Feng, S.1    Kroll, M.H.2    Nolasco, L.3
  • 63
    • 84856863401 scopus 로고    scopus 로고
    • Anovel C3 mutation causing increased formation of the C3 convertase in familial atypical hemolytic uremic syndrome
    • Sartz L., Olin A.I., Kristoffersson A.C., et al. Anovel C3 mutation causing increased formation of the C3 convertase in familial atypical hemolytic uremic syndrome. JImmunol 2012, 188(4):2030-2037.
    • (2012) JImmunol , vol.188 , Issue.4 , pp. 2030-2037
    • Sartz, L.1    Olin, A.I.2    Kristoffersson, A.C.3
  • 64
    • 67651166873 scopus 로고    scopus 로고
    • Thrombomodulin mutations in atypical hemolytic-uremic syndrome
    • Delvaeye M., Noris M., De Vriese A., et al. Thrombomodulin mutations in atypical hemolytic-uremic syndrome. NEngl J Med 2009, 361(4):345-357.
    • (2009) NEngl J Med , vol.361 , Issue.4 , pp. 345-357
    • Delvaeye, M.1    Noris, M.2    De Vriese, A.3
  • 65
    • 0014701847 scopus 로고
    • The production of schistocytes by fibrin strands (a scanning electron microscope study)
    • Bull B.S., Kuhn I.N. The production of schistocytes by fibrin strands (a scanning electron microscope study). Blood 1970, 35(1):104-111.
    • (1970) Blood , vol.35 , Issue.1 , pp. 104-111
    • Bull, B.S.1    Kuhn, I.N.2
  • 66
    • 57549087389 scopus 로고    scopus 로고
    • Glycosylphosphatidylinositol-anchored protein deficiency confers resistance to apoptosis in PNH
    • Savage W.J., Barber J.P., Mukhina G.L., et al. Glycosylphosphatidylinositol-anchored protein deficiency confers resistance to apoptosis in PNH. Exp Hematol 2009, 37(1):42-51.
    • (2009) Exp Hematol , vol.37 , Issue.1 , pp. 42-51
    • Savage, W.J.1    Barber, J.P.2    Mukhina, G.L.3
  • 67
    • 0025233715 scopus 로고
    • The complement-inhibitory activity of CD59 resides in its capacity to block incorporation of C9 into membrane C5b-9
    • Rollins S.A., Sims P.J. The complement-inhibitory activity of CD59 resides in its capacity to block incorporation of C9 into membrane C5b-9. JImmunol 1990, 144(9):3478-3483.
    • (1990) JImmunol , vol.144 , Issue.9 , pp. 3478-3483
    • Rollins, S.A.1    Sims, P.J.2
  • 68
    • 84896120944 scopus 로고    scopus 로고
    • Paroxysmal nocturnal haemoglobinuria. Experience over a 10 years period
    • Canalejo K., Riera Cervantes N., Felippo M., et al. Paroxysmal nocturnal haemoglobinuria. Experience over a 10 years period. Int J Lab Hematol 2014, 36(2):213-221.
    • (2014) Int J Lab Hematol , vol.36 , Issue.2 , pp. 213-221
    • Canalejo, K.1    Riera Cervantes, N.2    Felippo, M.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.