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Nephritic Factors: An Overview of Classification, Diagnostic Tools and Clinical Associations.

Corvillo F, Okrój M, Nozal P, Melgosa M, Sánchez-Corral P, López-Trascasa M.

Front Immunol. 2019 Apr 24;10:886. doi: 10.3389/fimmu.2019.00886. eCollection 2019. Review.


Potentiation of complement regulator factor H protects human endothelial cells from complement attack in aHUS sera.

Pouw RB, Brouwer MC, de Gast M, van Beek AE, van den Heuvel LP, Schmidt CQ, van der Ende A, Sánchez-Corral P, Kuijpers TW, Wouters D.

Blood Adv. 2019 Feb 26;3(4):621-632. doi: 10.1182/bloodadvances.2018025692.


Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family.

Sánchez-Corral P, Pouw RB, López-Trascasa M, Józsi M.

Front Immunol. 2018 Jul 12;9:1607. doi: 10.3389/fimmu.2018.01607. eCollection 2018. Review.


High Complement Factor H-Related (FHR)-3 Levels Are Associated With the Atypical Hemolytic-Uremic Syndrome-Risk Allele CFHR3*B.

Pouw RB, Gómez Delgado I, López Lera A, Rodríguez de Córdoba S, Wouters D, Kuijpers TW, Sánchez-Corral P.

Front Immunol. 2018 Apr 24;9:848. doi: 10.3389/fimmu.2018.00848. eCollection 2018.


Complement as a diagnostic tool in immunopathology.

López-Lera A, Corvillo F, Nozal P, Regueiro JR, Sánchez-Corral P, López-Trascasa M.

Semin Cell Dev Biol. 2019 Jan;85:86-97. doi: 10.1016/j.semcdb.2017.12.017. Epub 2018 Jan 12. Review.


Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome.

Nozal P, Bernabéu-Herrero ME, Uzonyi B, Szilágyi Á, Hyvärinen S, Prohászka Z, Jokiranta TS, Sánchez-Corral P, López-Trascasa M, Józsi M.

Mol Immunol. 2016 Feb;70:47-55. doi: 10.1016/j.molimm.2015.12.005. Epub 2015 Dec 15.


Complement factor H, FHR-3 and FHR-1 variants associate in an extended haplotype conferring increased risk of atypical hemolytic uremic syndrome.

Bernabéu-Herrero ME, Jiménez-Alcázar M, Anter J, Pinto S, Sánchez Chinchilla D, Garrido S, López-Trascasa M, Rodríguez de Córdoba S, Sánchez-Corral P.

Mol Immunol. 2015 Oct;67(2 Pt B):276-86. doi: 10.1016/j.molimm.2015.06.021. Epub 2015 Jul 7.


The molecular and structural bases for the association of complement C3 mutations with atypical hemolytic uremic syndrome.

Martínez-Barricarte R, Heurich M, López-Perrote A, Tortajada A, Pinto S, López-Trascasa M, Sánchez-Corral P, Morgan BP, Llorca O, Harris CL, Rodríguez de Córdoba S.

Mol Immunol. 2015 Aug;66(2):263-73. doi: 10.1016/j.molimm.2015.03.248. Epub 2015 Apr 11.


Atypical aHUS: State of the art.

Nester CM, Barbour T, de Cordoba SR, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship TH, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJ.

Mol Immunol. 2015 Sep;67(1):31-42. doi: 10.1016/j.molimm.2015.03.246. Epub 2015 Apr 3. Review.


Complement mutations in diacylglycerol kinase-ε-associated atypical hemolytic uremic syndrome.

Sánchez Chinchilla D, Pinto S, Hoppe B, Adragna M, Lopez L, Justa Roldan ML, Peña A, Lopez Trascasa M, Sánchez-Corral P, Rodríguez de Córdoba S.

Clin J Am Soc Nephrol. 2014 Sep 5;9(9):1611-9. doi: 10.2215/CJN.01640214. Epub 2014 Aug 18.


Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome.

Józsi M, Reuter S, Nozal P, López-Trascasa M, Sánchez-Corral P, Prohászka Z, Uzonyi B.

Immunol Lett. 2014 Aug;160(2):163-71. doi: 10.1016/j.imlet.2014.01.014. Epub 2014 Feb 1. Review.


An ELISA assay with two monoclonal antibodies allows the estimation of free factor H and identifies patients with acquired deficiency of this complement regulator.

Nozal P, Garrido S, Alba-Domínguez M, Espinosa L, Peña A, Córdoba SR, Sánchez-Corral P, López-Trascasa M.

Mol Immunol. 2014 Apr;58(2):194-200. doi: 10.1016/j.molimm.2013.11.021. Epub 2013 Dec 28.


Eculizumab long-term therapy for pediatric renal transplant in aHUS with CFH/CFHR1 hybrid gene.

Román-Ortiz E, Mendizabal Oteiza S, Pinto S, López-Trascasa M, Sánchez-Corral P, Rodríguez de Cordoba S.

Pediatr Nephrol. 2014 Jan;29(1):149-53. doi: 10.1007/s00467-013-2591-8. Epub 2013 Aug 24.


An engineered construct combining complement regulatory and surface-recognition domains represents a minimal-size functional factor H.

Hebecker M, Alba-Domínguez M, Roumenina LT, Reuter S, Hyvärinen S, Dragon-Durey MA, Jokiranta TS, Sánchez-Corral P, Józsi M.

J Immunol. 2013 Jul 15;191(2):912-21. doi: 10.4049/jimmunol.1300269. Epub 2013 Jun 14.


C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation.

Tortajada A, Yébenes H, Abarrategui-Garrido C, Anter J, García-Fernández JM, Martínez-Barricarte R, Alba-Domínguez M, Malik TH, Bedoya R, Cabrera Pérez R, López Trascasa M, Pickering MC, Harris CL, Sánchez-Corral P, Llorca O, Rodríguez de Córdoba S.

J Clin Invest. 2013 Jun;123(6):2434-46.


Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype.

Bresin E, Rurali E, Caprioli J, Sanchez-Corral P, Fremeaux-Bacchi V, Rodriguez de Cordoba S, Pinto S, Goodship TH, Alberti M, Ribes D, Valoti E, Remuzzi G, Noris M; European Working Party on Complement Genetics in Renal Diseases.

J Am Soc Nephrol. 2013 Feb;24(3):475-86. doi: 10.1681/ASN.2012090884. Epub 2013 Feb 21.


Atypical hemolytic uremic syndrome-associated variants and autoantibodies impair binding of factor h and factor h-related protein 1 to pentraxin 3.

Kopp A, Strobel S, Tortajada A, Rodríguez de Córdoba S, Sánchez-Corral P, Prohászka Z, López-Trascasa M, Józsi M.

J Immunol. 2012 Aug 15;189(4):1858-67. doi: 10.4049/jimmunol.1200357. Epub 2012 Jul 11.


Anti-factor H antibody affecting factor H cofactor activity in a patient with dense deposit disease.

Nozal P, Strobel S, Ibernon M, López D, Sánchez-Corral P, Rodríguez de Córdoba S, Józsi M, López-Trascasa M.

Clin Kidney J. 2012 Apr;5(2):133-136. doi: 10.1093/ckj/sfs002. Epub 2012 Mar 15.


Early treatment with eculizumab in atypical haemolytic uraemic syndrome.

Garjau M, Azancot M, Ramos R, Sánchez-Corral P, Montero MA, Serón D.

Clin Kidney J. 2012 Feb;5(1):31-3. doi: 10.1093/ndtplus/sfr157. Epub 2012 Jan 31.


Factor H and CFHR1 polymorphisms associated with atypical Haemolytic Uraemic Syndrome (aHUS) are differently expressed in Tunisian and in Caucasian populations.

Leban N, Abarrategui-Garrido C, Fariza-Requejo E, Amiñoso-Carbonero C, Pinto S, Chibani JB, Khelil AH, Sánchez-Corral P.

Int J Immunogenet. 2012 Apr;39(2):110-3. doi: 10.1111/j.1744-313X.2011.01071.x. Epub 2011 Dec 5.


Complement factor H variants I890 and L1007 while commonly associated with atypical hemolytic uremic syndrome are polymorphisms with no functional significance.

Tortajada A, Pinto S, Martínez-Ara J, López-Trascasa M, Sánchez-Corral P, de Córdoba SR.

Kidney Int. 2012 Jan;81(1):56-63. doi: 10.1038/ki.2011.291. Epub 2011 Aug 31.


Factor H-related protein 1 neutralizes anti-factor H autoantibodies in autoimmune hemolytic uremic syndrome.

Strobel S, Abarrategui-Garrido C, Fariza-Requejo E, Seeberger H, Sánchez-Corral P, Józsi M.

Kidney Int. 2011 Aug;80(4):397-404. doi: 10.1038/ki.2011.152. Epub 2011 Jun 15.


Advances in understanding the aetiology of atypical Haemolytic Uraemic Syndrome.

Sánchez-Corral P, Melgosa M.

Br J Haematol. 2010 Sep;150(5):529-42. doi: 10.1111/j.1365-2141.2010.08295.x. Epub 2010 Jul 14. Review.


Atypical hemolytic uremic syndrome in the Tunisian population.

Leban N, Aloui S, Touati D, Lakhdhar R, Skhiri H, Lefranc G, Achour A, Elmay M, Lopez-Trascasa M, Sanchez-Corral P, Chibani J, Haj Khelil A.

Int Urol Nephrol. 2011 Jun;43(2):559-64. doi: 10.1007/s11255-010-9754-3. Epub 2010 May 25.


Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome.

Abarrategui-Garrido C, Martínez-Barricarte R, López-Trascasa M, de Córdoba SR, Sánchez-Corral P.

Blood. 2009 Nov 5;114(19):4261-71. doi: 10.1182/blood-2009-05-223834. Epub 2009 Sep 10.


Successful renal transplantation in a patient with atypical hemolytic uremic syndrome carrying mutations in both factor I and MCP.

Cruzado JM, de Córdoba SR, Melilli E, Bestard O, Rama I, Sánchez-Corral P, López-Trascasa M, Navarro I, Torras J, Gomà M, Grinyó JM.

Am J Transplant. 2009 Jun;9(6):1477-83. doi: 10.1111/j.1600-6143.2009.02647.x. Epub 2009 May 20.


Mutations in proteins of the alternative pathway of complement and the pathogenesis of atypical hemolytic uremic syndrome.

Abarrategui-Garrido C, Melgosa M, Peña-Carrión A, de Jorge EG, de Córdoba SR, López-Trascasa M, Sánchez-Corral P.

Am J Kidney Dis. 2008 Jul;52(1):171-80. doi: 10.1053/j.ajkd.2008.01.026. Epub 2008 Apr 18.


Molecular characterization of Complement Factor I deficiency in two Spanish families.

Ponce-Castro IM, González-Rubio C, Delgado-Cerviño EM, Abarrategui-Garrido C, Fontán G, Sánchez-Corral P, López-Trascasa M.

Mol Immunol. 2008 May;45(10):2764-71. doi: 10.1016/j.molimm.2008.02.008. Epub 2008 Mar 28.


Genetic deficiency of complement factor H in a patient with age-related macular degeneration and membranoproliferative glomerulonephritis.

Montes T, Goicoechea de Jorge E, Ramos R, Gomà M, Pujol O, Sánchez-Corral P, Rodríguez de Córdoba S.

Mol Immunol. 2008 May;45(10):2897-904. doi: 10.1016/j.molimm.2008.01.027. Epub 2008 Mar 12.


Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome.

Goicoechea de Jorge E, Harris CL, Esparza-Gordillo J, Carreras L, Arranz EA, Garrido CA, López-Trascasa M, Sánchez-Corral P, Morgan BP, Rodríguez de Córdoba S.

Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):240-5. Epub 2006 Dec 20. Erratum in: Proc Natl Acad Sci U S A. 2007 Jun 19;104(25):10749.


The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models.

Saunders RE, Abarrategui-Garrido C, Frémeaux-Bacchi V, Goicoechea de Jorge E, Goodship TH, López Trascasa M, Noris M, Ponce Castro IM, Remuzzi G, Rodríguez de Córdoba S, Sánchez-Corral P, Skerka C, Zipfel PF, Perkins SJ.

Hum Mutat. 2007 Mar;28(3):222-34.


De novo gene conversion in the RCA gene cluster (1q32) causes mutations in complement factor H associated with atypical hemolytic uremic syndrome.

Heinen S, Sanchez-Corral P, Jackson MS, Strain L, Goodship JA, Kemp EJ, Skerka C, Jokiranta TS, Meyers K, Wagner E, Robitaille P, Esparza-Gordillo J, Rodriguez de Cordoba S, Zipfel PF, Goodship TH.

Hum Mutat. 2006 Mar;27(3):292-3.


Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree.

Esparza-Gordillo J, Jorge EG, Garrido CA, Carreras L, López-Trascasa M, Sánchez-Corral P, de Córdoba SR.

Mol Immunol. 2006 Apr;43(11):1769-75. Epub 2006 Jan 18.


Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.

Esparza-Gordillo J, Goicoechea de Jorge E, Buil A, Carreras Berges L, López-Trascasa M, Sánchez-Corral P, Rodríguez de Córdoba S.

Hum Mol Genet. 2005 Mar 1;14(5):703-12. Epub 2005 Jan 20. Erratum in: Hum Mol Genet. 2005 Apr 15;14(8):1107.


The human complement factor H: functional roles, genetic variations and disease associations.

Rodríguez de Córdoba S, Esparza-Gordillo J, Goicoechea de Jorge E, Lopez-Trascasa M, Sánchez-Corral P.

Mol Immunol. 2004 Jun;41(4):355-67. Review.


Functional analysis in serum from atypical Hemolytic Uremic Syndrome patients reveals impaired protection of host cells associated with mutations in factor H.

Sánchez-Corral P, González-Rubio C, Rodríguez de Córdoba S, López-Trascasa M.

Mol Immunol. 2004 May;41(1):81-4.


[Genetic factors in the haemolytic uremic syndrome. Diagnostic and therapeutic implications].

Rodríguez de Córdoba S, Peña A, Rivera F, López Trascasa M, Sánchez-Corral P.

Nefrologia. 2004;24(1):9-11. Spanish. No abstract available.


Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome.

Sánchez-Corral P, Pérez-Caballero D, Huarte O, Simckes AM, Goicoechea E, López-Trascasa M, de Córdoba SR.

Am J Hum Genet. 2002 Dec;71(6):1285-95. Epub 2002 Nov 6.


Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome.

Pérez-Caballero D, González-Rubio C, Gallardo ME, Vera M, López-Trascasa M, Rodríguez de Córdoba S, Sánchez-Corral P.

Am J Hum Genet. 2001 Feb;68(2):478-84. Epub 2001 Jan 17.


Molecular basis for factor H and FHL-1 deficiency in an Italian family.

Sánchez-Corral P, Bellavia D, Amico L, Brai M, Rodríguez de Córdoba S.

Immunogenetics. 2000 Apr;51(4-5):366-9. No abstract available.


Assessment of the interaction of human complement regulatory proteins with group A Streptococcus. Identification of a high-affinity group A Streptococcus binding site in FHL-1.

Pérez-Caballero D, Albertí S, Vivanco F, Sánchez-Corral P, Rodríguez de Córdoba S.

Eur J Immunol. 2000 Apr;30(4):1243-53.


Binding of human complement component C4b-binding protein (C4BP) to Streptococcus pyogenes involves the C4b-binding site.

Accardo P, Sánchez-Corral P, Criado O, García E, Rodríguez de Córdoba S.

J Immunol. 1996 Dec 1;157(11):4935-9.


Isoforms of human C4b-binding protein. II. Differential modulation of the C4BPA and C4BPB genes by acute phase cytokines.

Criado García O, Sánchez-Corral P, Rodríguez de Córdoba S.

J Immunol. 1995 Oct 15;155(8):4037-43.


Isoforms of human C4b-binding protein. I. Molecular basis for the C4BP isoform pattern and its variations in human plasma.

Sánchez-Corral P, Criado García O, Rodríguez de Córdoba S.

J Immunol. 1995 Oct 15;155(8):4030-6.


The gene coding for the beta-chain of C4b-binding protein (C4BPB) has become a pseudogene in the mouse.

Rodríguez de Córdoba S, Pérez-Blas M, Ramos-Ruiz R, Sánchez-Corral P, Pardo-Manuel de Villena F, Rey-Campos J.

Genomics. 1994 Jun;21(3):501-9.


C4BPAL1, a member of the human regulator of complement activation (RCA) gene cluster that resulted from the duplication of the gene coding for the alpha-chain of C4b-binding protein.

Sánchez-Corral P, Pardo-Manuel de Villena F, Rey-Campos J, Rodríguez de Córdoba S.

Genomics. 1993 Jul;17(1):185-93.


Dinucleotide repeat polymorphism between the human C4BPA and C4BPB gene loci (1q32).

Velasco E, Sanchez-Corral P, Moreno F, Rodriguez de Cordoba S.

Hum Mol Genet. 1992 Oct;1(7):552. No abstract available.


Structure of the gene coding for the alpha polypeptide chain of the human complement component C4b-binding protein.

Rodriguez de Cordoba S, Sanchez-Corral P, Rey-Campos J.

J Exp Med. 1991 May 1;173(5):1073-82.


Proteolytic activity of the different fragments of factor B on the third component of complement (C3). Involvement of the N-terminal domain of Bb in magnesium binding.

Sánchez-Corral P, Antón LC, Alcolea JM, Marqués G, Sánchez A, Vivanco F.

Mol Immunol. 1990 Sep;27(9):891-900.


Separation of active and inactive forms of the third component of human complement, C3, by fast protein liquid chromatography (FPLC).

Sánchez-Corral P, Antón LC, Alcolea JM, Marqués G, Sánchez A, Vivanco F.

J Immunol Methods. 1989 Aug 15;122(1):105-13.


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