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List of works by Santiago Rodriguez de Córdoba

3D structure of the C3bB complex provides insights into the activation and regulation of the complement alternative pathway convertase.

scholarly article

A high-resolution map of the regulator of the complement activation gene cluster on 1q32 that integrates new genes and markers

article

A novel antibody against human factor B that blocks formation of the C3bB proconvertase and inhibits complement activation in disease models.

scientific article

A novel atypical hemolytic uremic syndrome-associated hybrid CFHR1/CFH gene encoding a fusion protein that antagonizes factor H-dependent complement regulation

scientific article

A radiation hybrid map of complement factor H and factor H-related genes

scientific article (publication date: June 1999)

A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome.

scientific article published on 11 September 2017

AMP-activated protein kinase phosphorylates R5/PTG, the glycogen targeting subunit of the R5/PTG-protein phosphatase 1 holoenzyme, and accelerates its down-regulation by the laforin-malin complex

scientific article

Abnormal glycogen chain length pattern, not hyperphosphorylation, is critical in Lafora disease.

scientific article

Absence of CD59 in Guinea Pigs: Analysis of the Cavia porcellus Genome Suggests the Evolution of a CD59 Pseudogene.

scientific article

An integrated map of the human regulator of complement activation (RCA) gene cluster on 1q32

article

An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document

scientific article

Analysis of alkaptonuria (AKU) mutations and polymorphisms reveals that the CCC sequence motif is a mutational hot spot in the homogentisate 1,2 dioxygenase gene (HGO).

scientific article published in May 1999

Anti-C5 as prophylactic therapy in atypical hemolytic uremic syndrome in living-related kidney transplantation

scientific article published in August 2013

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

scientific article published on 15 March 2012

Antibody directs properdin-dependent activation of the complement alternative pathway in a mouse model of abdominal aortic aneurysm

scientific article published on 30 January 2012

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.

scientific article published in April 2000

Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

scientific article published on 15 December 2016

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

scientific article

Binding of complement regulatory proteins to group A Streptococcus

scientific article published on December 2008

C3 glomerulopathy - understanding a rare complement-driven renal disease

scientific article published on 01 March 2019

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

scientific article published on June 2013

C3 glomerulopathy: consensus report

scientific article published on 30 October 2013

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

scientific article published on July 1993

C4BPAL2: a second duplication of the C4BPA gene in the human RCA gene cluster

scientific article published in January 1995

C4BPB/C4BPA is a new susceptibility locus for venous thrombosis with unknown protein S-independent mechanism: results from genome-wide association and gene expression analyses followed by case-control studies

scientific article

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

scientific article

Cloning, characterization and chromosome mapping of the human SMAP1 gene

scientific article

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

scientific article

Coagulation factor XIII B subunit is encoded by a gene linked to the regulator of complement activation (RCA) gene cluster in man

article

Coexistence of closed and open conformations of complement factor B in the alternative pathway C3bB(Mg2+) proconvertase

scientific article published on 4 November 2009

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

scientific article

Common and rare genetic variants of complement components in human disease

scientific article published on 18 June 2018

Common polymorphisms in C3, factor B, and factor H collaborate to determine systemic complement activity and disease risk

scientific article

Complement analysis in the 21st century

scientific article

Complement dysregulation and disease: From genes and proteins to diagnostics and drugs ⬇️

scientific article published on November 1, 2012

Complement factor H binds to denatured rather than to native pentameric C-reactive protein

scientific article published on 11 September 2008

Complement factor H is expressed in adipose tissue in association with insulin resistance

scientific article

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

scientific article

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

scientific article

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

scientific article

Complete functional characterization of disease-associated genetic variants in the complement factor H gene.

scientific article

Crystal Structure of Glyceraldehyde-3-Phosphate Dehydrogenase from the Gram-Positive Bacterial Pathogen A. vaginae, an Immunoevasive Factor that Interacts with the Human C5a Anaphylatoxin

scientific article published on 10 April 2017

Crystal structure of human homogentisate dioxygenase

scientific article published in July 2000

Cytokine-mediated up-regulation of CD55 and CD59 protects human hepatoma cells from complement attack.

scientific article published on August 2000

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

scientific article

Definition of IDDM-associated HLA DQ and DX RFLPs by segregation analysis of multiplex sibships

scientific article published on 01 January 1989

Differences in reactive oxygen species production explain the phenotypes associated with common mouse mitochondrial DNA variants

scientific article published on October 2006

Does the mouse C4-binding protein gene (C4BP) map in the H-2 region?

article

Eculizumab Modifies Outcomes in Adults with Atypical Hemolytic Uremic Syndrome with Acute Kidney Injury

scientific article published on 11 September 2018

Eculizumab in dense-deposit disease after renal transplantation

scientific article published on 08 June 2014

Eculizumab in secondary atypical haemolytic uraemic syndrome

scientific article

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

scientific article

Elevated factor H-related protein 1 and factor H pathogenic variants decrease complement regulation in IgA nephropathy.

scientific article published on 18 June 2017

Erratum to: Knecht E, Criado-García O, Aguado C, Gayarre J, Duran-Trio L, Garcia-Cabrero AM, et al. Malin knockout mice support a primary role of autophagy in the pathogenesis of Lafora disease. Autophagy 2012; 8.

scientific article published in July 2012

Evidence for linkage between the loci coding for the binding protein for the fourth component of human complement (C4BP) and for the C3b/C4b receptor

scientific article published on December 1984

Extravascular hemolysis and complement consumption in Paroxysmal Nocturnal Hemoglobinuria patients undergoing eculizumab treatment

scientific article published on 13 September 2016

FHR-1 Binds to C-Reactive Protein and Enhances Rather than Inhibits Complement Activation.

scientific article

Factor H Competitor Generated by Gene Conversion Events Associates with Atypical Hemolytic Uremic Syndrome

scientific article

Factor H-related proteins determine complement-activating surfaces

scientific article published on 13 May 2015

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

scientific article

Functional analysis of MCCA and MCCB mutations causing methylcrotonylglycinuria.

scientific article published in November 2003

Functional and structural characterization of four mouse monoclonal antibodies to complement C3 with potential therapeutic and diagnostic applications.

scientific article published on 13 January 2017

Functional basis of protection against age-related macular degeneration conferred by a common polymorphism in complement factor B

scholarly article

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

scientific article

Genetic and environmental factors influencing the human factor H plasma levels

scientific article

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

scientific article published on 12 March 2008

Genetics of atypical hemolytic uremic syndrome (aHUS).

scientific article

Genomic cloning, structure, expression pattern, and chromosomal location of the human SIX3 gene.

scientific article

Genomic sequence of the pathogenic and allergenic filamentous fungus Aspergillus fumigatus

scientific article

Genética y DMAE: del laboratorio a la consulta

scientific article published on 01 April 2011

Guidelines for the use and interpretation of assays for monitoring autophagy

scientific article

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

scientific article

HLA antigens in a sample of the Spanish population: common features among Spaniards, Basques, and Sardinians

scientific article

Hepatic disease as the first manifestation of progressive myoclonus epilepsy of Lafora

scientific article

High resolution isoelectric focusing of immunoprecipitated proteins under denaturing conditions. A simple analytical method applied to the study of complement component polymorphisms

scientific article published on 01 April 1984

How novel structures inform understanding of complement function

scientific article published on 14 August 2017

Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation

scientific article

Human T-cell lymphotropic virus type III infection in a cohort of homosexual men in New York City

scientific article published in April 1986

Human genes for the alpha and beta chains of complement C4b-binding protein are closely linked in a head-to-tail arrangement

scientific article

Human plasma C3 is essential for the development of memory B, but not T, lymphocytes.

scientific article

Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis

scientific article

Impaired autophagy in Lafora disease

scientific article

Increased endoplasmic reticulum stress and decreased proteasomal function in lafora disease models lacking the phosphatase laforin

scientific article

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

scientific article

Interaction of Shiga toxin 2 with complement regulators of the factor H protein family

scientific article

Intracellular complement activation sustains T cell homeostasis and mediates effector differentiation

scientific article published on 05 December 2013

Ionic tethering contributes to the conformational stability and function of complement C3b.

scientific article published on 27 February 2017

Kidney, hypertension and complement activation. In search of new therapeutic targets

scientific article published on 07 December 2018

Lack of association between polymorphisms in C4b-binding protein and atypical haemolytic uraemic syndrome in the Spanish population

scientific article

Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy

scientific article published on 20 December 2011

Laforin, a dual specificity phosphatase involved in Lafora disease, regulates insulin response and whole-body energy balance in mice.

scientific article published on 14 April 2011

Laforin, a dual-specificity phosphatase involved in Lafora disease, is phosphorylated at Ser25 by AMP-activated protein kinase.

scientific article published in October 2011

Laforin, the dual-phosphatase responsible for Lafora disease, interacts with R5 (PTG), a regulatory subunit of protein phosphatase-1 that enhances glycogen accumulation

scientific article

Laforin, the most common protein mutated in Lafora disease, regulates autophagy

scientific article

Lessons from functional and structural analyses of disease-associated genetic variants in the complement alternative pathway.

scientific article

Malin knockout mice support a primary role of autophagy in the pathogenesis of Lafora disease

scientific article published on April 2012

Measurement of factor H variants in plasma using variant-specific monoclonal antibodies: application to assessing risk of age-related macular degeneration

scientific article

Mechanism suppressing glycogen synthesis in neurons and its demise in progressive myoclonus epilepsy.

scientific article published on 21 October 2007

Membrane cofactor protein (MCP, CD46) binding to clinical isolates of Streptococcus pyogenes: binding to M type 18 strains is independent of Emm or Enn proteins

scientific article published on 30 April 2007

Molecular analyses of the HGO gene mutations in Turkish alkaptonuria patients suggest that the R58fs mutation originated from central Asia and was spread throughout Europe and Anatolia by human migrations

scientific article

Molecular characterization by high-resolution isoelectric focusing of the products encoded by the class II region loci of the major histocompatibility complex in humans. I. DR and DQ gene variants

article

Mutational spectrum of the EPM2A gene in progressive myoclonus epilepsy of Lafora: high degree of allelic heterogeneity and prevalence of deletions

article

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

scientific article

New alleles of C4-binding protein and factor H and further linkage data in the regulator of complement activation (RCA) gene cluster in man.

scientific article

New approaches to the treatment of dense deposit disease

scientific article published on 5 August 2007

Ochronotic rheumatism in Algeria: clinical, radiological, biological and molecular studies--a case study of 14 patients in 11 families

scientific article published on 7 July 2005

Physical linkage of the human genes coding for complement factor H and coagulation factor XIII B subunit

article

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

scientific article

Regulation of glycogen synthesis by the laforin-malin complex is modulated by the AMP-activated protein kinase pathway

scientific article published on 20 November 2007

Relevance of complement factor H-related 1 (CFHR1) genotypes in age-related macular degeneration.

scientific article published in March 2012

Reply to “Reactive oxygen species and the segregation of mtDNA sequence variants”

Secondary atypical hemolytic uremic syndromes in the era of complement blockade

scientific article published on 01 June 2019

Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy

scientific article

Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome

scientific article published on 31 May 2019

Six9 (Optx2), a new member of the six gene family of transcription factors, is expressed at early stages of vertebrate ocular and pituitary development

scientific article

Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains

scientific article

Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy.

scientific article published on 2 March 2018

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

scientific article

Structural basis for the stabilization of the complement alternative pathway C3 convertase by properdin.

scientific article

Testing the Activity of Complement Convertases in Serum/Plasma for Diagnosis of C4NeF-Mediated C3 Glomerulonephritis.

scientific article published on 05 May 2016

The 7 0 Isoform of the Complement Regulator C4b-Binding Protein Induces a Semimature, Anti-Inflammatory State in Dendritic Cells

scientific article published on 06 February 2013

The Antimicrobials Anacardic Acid and Curcumin Are Not-Competitive Inhibitors of Gram-Positive Bacterial Pathogenic Glyceraldehyde-3-Phosphate Dehydrogenase by a Mechanism Unrelated to Human C5a Anaphylatoxin Binding

scientific article published on 26 February 2019

The Complement Inhibitor Factor H Generates an Anti-Inflammatory and Tolerogenic State in Monocyte-Derived Dendritic Cells

scientific article published on 13 April 2016

The complement factor H R1210C mutation is associated with atypical hemolytic uremic syndrome

scientific article

The crystal structure of iC3b-CR3 αI reveals a modular recognition of the main opsonin iC3b by the CR3 integrin receptor

scientific article published on 12 April 2022

The familial risk of developing atypical Hemolytic Uremic Syndrome

scientific article published on 02 June 2020

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

scientific article published on June 1994

The human homogentisate 1,2-dioxygenase (HGO) gene

scientific article published in July 1997

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

scientific article

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

scientific article

The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease

scientific article published on 14 January 2014

Twenty-six DRβ, and 16 DQβ chain IEF variants and their associated HLA-DR, HLA-DQ, and HLA-Dw specificities

scientific article published on 01 January 1989

Unique structure of iC3b resolved at a resolution of 24 Å by 3D-electron microscopy

scientific article

Variant-specific quantification of factor H in plasma identifies null alleles associated with atypical hemolytic uremic syndrome

scientific article

aHUS: a disorder with many risk factors

scientific article published on 01 January 2010

m.6267G>A: a recurrent mutation in the human mitochondrial DNA that reduces cytochrome c oxidase activity and is associated with tumors

scientific article published on June 2006

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