Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome
The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Wong, Edwin K.S. [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2015transfer abstract |
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| Schlagwörter: |
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| Umfang: |
15 |
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| Übergeordnetes Werk: |
Enthalten in: Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties - Muthuraja, A. ELSEVIER, 2015, the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research, New York, NY |
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| Übergeordnetes Werk: |
volume:165 ; year:2015 ; number:2 ; pages:306-320 ; extent:15 |
| Links: |
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| DOI / URN: |
10.1016/j.trsl.2014.10.010 |
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| 520 | |a The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. | ||
| 520 | |a The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. | ||
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10.1016/j.trsl.2014.10.010 doi GBVA2015011000021.pica (DE-627)ELV034563296 (ELSEVIER)S1931-5244(14)00383-1 DE-627 ger DE-627 rakwb eng 610 610 DE-600 530 VZ 620 VZ 670 VZ 300 VZ 70.00 bkl 71.00 bkl Wong, Edwin K.S. verfasserin aut Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome 2015transfer abstract 15 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. GPI Elsevier NO Elsevier FH Elsevier PEX Elsevier FI Elsevier aHUS Elsevier IgG Elsevier PNH Elsevier LDH Elsevier TTP Elsevier DGKε Elsevier SNP Elsevier Stx Elsevier PIGA Elsevier CKD Elsevier CD55 Elsevier CR1 Elsevier AE Elsevier TMA Elsevier GAG Elsevier CP Elsevier GPI-AP Elsevier MAC Elsevier AP Elsevier C3bBb Elsevier FB Elsevier CD46 Elsevier FD Elsevier Kavanagh, David oth Enthalten in Elsevier Muthuraja, A. ELSEVIER Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties 2015 the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research New York, NY (DE-627)ELV013179047 volume:165 year:2015 number:2 pages:306-320 extent:15 https://doi.org/10.1016/j.trsl.2014.10.010 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U 70.00 Sozialwissenschaften allgemein: Allgemeines VZ 71.00 Soziologie: Allgemeines VZ AR 165 2015 2 306-320 15 045F 610 |
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10.1016/j.trsl.2014.10.010 doi GBVA2015011000021.pica (DE-627)ELV034563296 (ELSEVIER)S1931-5244(14)00383-1 DE-627 ger DE-627 rakwb eng 610 610 DE-600 530 VZ 620 VZ 670 VZ 300 VZ 70.00 bkl 71.00 bkl Wong, Edwin K.S. verfasserin aut Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome 2015transfer abstract 15 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. GPI Elsevier NO Elsevier FH Elsevier PEX Elsevier FI Elsevier aHUS Elsevier IgG Elsevier PNH Elsevier LDH Elsevier TTP Elsevier DGKε Elsevier SNP Elsevier Stx Elsevier PIGA Elsevier CKD Elsevier CD55 Elsevier CR1 Elsevier AE Elsevier TMA Elsevier GAG Elsevier CP Elsevier GPI-AP Elsevier MAC Elsevier AP Elsevier C3bBb Elsevier FB Elsevier CD46 Elsevier FD Elsevier Kavanagh, David oth Enthalten in Elsevier Muthuraja, A. ELSEVIER Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties 2015 the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research New York, NY (DE-627)ELV013179047 volume:165 year:2015 number:2 pages:306-320 extent:15 https://doi.org/10.1016/j.trsl.2014.10.010 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U 70.00 Sozialwissenschaften allgemein: Allgemeines VZ 71.00 Soziologie: Allgemeines VZ AR 165 2015 2 306-320 15 045F 610 |
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10.1016/j.trsl.2014.10.010 doi GBVA2015011000021.pica (DE-627)ELV034563296 (ELSEVIER)S1931-5244(14)00383-1 DE-627 ger DE-627 rakwb eng 610 610 DE-600 530 VZ 620 VZ 670 VZ 300 VZ 70.00 bkl 71.00 bkl Wong, Edwin K.S. verfasserin aut Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome 2015transfer abstract 15 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. GPI Elsevier NO Elsevier FH Elsevier PEX Elsevier FI Elsevier aHUS Elsevier IgG Elsevier PNH Elsevier LDH Elsevier TTP Elsevier DGKε Elsevier SNP Elsevier Stx Elsevier PIGA Elsevier CKD Elsevier CD55 Elsevier CR1 Elsevier AE Elsevier TMA Elsevier GAG Elsevier CP Elsevier GPI-AP Elsevier MAC Elsevier AP Elsevier C3bBb Elsevier FB Elsevier CD46 Elsevier FD Elsevier Kavanagh, David oth Enthalten in Elsevier Muthuraja, A. ELSEVIER Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties 2015 the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research New York, NY (DE-627)ELV013179047 volume:165 year:2015 number:2 pages:306-320 extent:15 https://doi.org/10.1016/j.trsl.2014.10.010 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U 70.00 Sozialwissenschaften allgemein: Allgemeines VZ 71.00 Soziologie: Allgemeines VZ AR 165 2015 2 306-320 15 045F 610 |
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10.1016/j.trsl.2014.10.010 doi GBVA2015011000021.pica (DE-627)ELV034563296 (ELSEVIER)S1931-5244(14)00383-1 DE-627 ger DE-627 rakwb eng 610 610 DE-600 530 VZ 620 VZ 670 VZ 300 VZ 70.00 bkl 71.00 bkl Wong, Edwin K.S. verfasserin aut Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome 2015transfer abstract 15 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. GPI Elsevier NO Elsevier FH Elsevier PEX Elsevier FI Elsevier aHUS Elsevier IgG Elsevier PNH Elsevier LDH Elsevier TTP Elsevier DGKε Elsevier SNP Elsevier Stx Elsevier PIGA Elsevier CKD Elsevier CD55 Elsevier CR1 Elsevier AE Elsevier TMA Elsevier GAG Elsevier CP Elsevier GPI-AP Elsevier MAC Elsevier AP Elsevier C3bBb Elsevier FB Elsevier CD46 Elsevier FD Elsevier Kavanagh, David oth Enthalten in Elsevier Muthuraja, A. ELSEVIER Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties 2015 the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research New York, NY (DE-627)ELV013179047 volume:165 year:2015 number:2 pages:306-320 extent:15 https://doi.org/10.1016/j.trsl.2014.10.010 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U 70.00 Sozialwissenschaften allgemein: Allgemeines VZ 71.00 Soziologie: Allgemeines VZ AR 165 2015 2 306-320 15 045F 610 |
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10.1016/j.trsl.2014.10.010 doi GBVA2015011000021.pica (DE-627)ELV034563296 (ELSEVIER)S1931-5244(14)00383-1 DE-627 ger DE-627 rakwb eng 610 610 DE-600 530 VZ 620 VZ 670 VZ 300 VZ 70.00 bkl 71.00 bkl Wong, Edwin K.S. verfasserin aut Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome 2015transfer abstract 15 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. GPI Elsevier NO Elsevier FH Elsevier PEX Elsevier FI Elsevier aHUS Elsevier IgG Elsevier PNH Elsevier LDH Elsevier TTP Elsevier DGKε Elsevier SNP Elsevier Stx Elsevier PIGA Elsevier CKD Elsevier CD55 Elsevier CR1 Elsevier AE Elsevier TMA Elsevier GAG Elsevier CP Elsevier GPI-AP Elsevier MAC Elsevier AP Elsevier C3bBb Elsevier FB Elsevier CD46 Elsevier FD Elsevier Kavanagh, David oth Enthalten in Elsevier Muthuraja, A. ELSEVIER Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties 2015 the journal of laboratory and clinical medicine : the official publication of the Central Society for Clinical Research New York, NY (DE-627)ELV013179047 volume:165 year:2015 number:2 pages:306-320 extent:15 https://doi.org/10.1016/j.trsl.2014.10.010 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U 70.00 Sozialwissenschaften allgemein: Allgemeines VZ 71.00 Soziologie: Allgemeines VZ AR 165 2015 2 306-320 15 045F 610 |
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Enthalten in Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties New York, NY volume:165 year:2015 number:2 pages:306-320 extent:15 |
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Enthalten in Growth of organic benzimidazole (BMZ) single crystal by vertical Bridgman technique and its structural, spectral, thermal, optical, mechanical and dielectric properties New York, NY volume:165 year:2015 number:2 pages:306-320 extent:15 |
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Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome |
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The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. |
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The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. |
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The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. |
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In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS.</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. 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