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Most relevant scientific articles
• subías HiDalGo m., tortaJaDa a., GastolDi s., Galbusera m., lópez-perrote a., De Juana lópez l., González- FernánDez Fa., VilleGas-martínez a., DomínGuez m., llorca o., noris m., morGan pb . and Rodríguez de Córdoba S . A novel antibody against human factor B that blocks formation of the C3bB proconvertase and inhibits complement activation in disease models . J . Immunol . 193(11):5567-75 (2014)
• sáncHez-cHincHilla D.#, pinto s.#, Hoppe b., aDraGna m., lópez l., Justa rolDan ml., peña a., lópez-trascasa m., sáncHez-corral p. anD roDríGuez De córDoba s . Complement Mutations in Diacylglycerol Kinase-e- Associated atypical Hemolytic Uremic Syndrome . Clin J Am Soc Nephrol . 9:1611-1619 (2014) (# Equal contribution authors)
• sáncHez-moreno a., De la cerDa F., cabrera r., FiJo J., lópez-trascasa m., beDoya r., roDríGuez De córDoba s . and Ybot-González P . Eculizumab in Dense-Deposit Disease after renal transplantation . Pediatr . Nephrol . 29:2055-2059 (2014)
• roDríGuez De córDoba s., subías-HiDalGo m., pinto s. anD tortaJaDa a . Genetics of atypical Hemolytic Ure- mic Syndrome (aHUS) . Semin Thromb . Hemost . 40:422-430 (2014)
• Gayarre J., Durán-trío l., criaDo García o., aGuaDo c., Juana-lópez l., crespo i., KnecHt e., boVolenta p. anD roDríGuez De córDoba s . The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease . Brain . 137:806-18 (2014)
Highlights
Institution: Agencia Estatal Consejo Superior de Investigaciones Científicas Contact: Centro de Investigaciones Biológicas . C/ Ramiro de Maeztu, 9 . 28040 Madrid · Tel .: (+34) 91 837 31 12 x4432/3 E .mail: srdecordoba@cib .csic .es · http://www .cib .csic .es/es/grupo .php?idgrupo=21
Our research and translational activity focus in the study of rare diseases associated with complement dysregulation and in Lafora disease . Our 2014 contributions, identifying new genetic associations or characterizing novel pathogenic mechanisms, represent important advances in our understanding of these disorders and provide new therapeutic possibilities . In different reviews and consensus reports published, or submitted for publication, during this period we have summarized our opinion in relation to the association between complement and disease, highlighting the importance contribution that complement dysregulation plays in diseases like atypical Uremic Hemolytic Syndrome and C3-glomerulopathies and how this improved knowledge of rare diseases have also important consequences in prevalent diseases like Age-related Macular Degeneration and IgA Nephropathy . During 2014 our laboratory lectured 25 educational talks or seminars to different clinical groups (national and international), where we emphasized these important advances in the complement field and the usefulness of this knowledge in the clinical practice . During 2014 we have continue developing diagnostics strategies, including, a CGH array for the detection of CNV in complement genes and a platform for the screening by NGS of the genes associated with aHUS .
Also, we begin a research study in order to generate complement inhibitors with therapeutic interest, which has already generated a patent for an anti-FB antibody . Our group is an international reference in the physiopathology of the complement system and a very important asset for the Spanish’s health public system . We develop a very strong translational activity in different medical specialties like nephrology, ophthalmology and hematology, providing to many patients (more than 88 during 2014) with a genetic and molecular analysis of the complement system and specific suggestions related to their treatments . Also of strategic interest is the registry of patients with renal pathology that we have developed with the supervision and support of CIBERER .
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