Santiago Rodrı́guez de Córdoba
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View article: C3 mutations and poor pegcetacoplan response in paroxysmal nocturnal hemoglobinuria
C3 mutations and poor pegcetacoplan response in paroxysmal nocturnal hemoglobinuria Open
Introduction Paroxysmal nocturnal hemoglobinuria (PNH) is treated with complement inhibitors, yet incomplete responses remain a challenge. Terminal inhibition with eculizumab prevents intravascular hemolysis but often leaves residual extra…
View article: Statistical interpretation of cases involving mixtures: A Spanish and Portuguese-Speaking Working Group (GHEP-ISFG) collaborative exercise
Statistical interpretation of cases involving mixtures: A Spanish and Portuguese-Speaking Working Group (GHEP-ISFG) collaborative exercise Open
The increasing complexity of forensic genetic samples has driven the development and implementation of probabilistic genotyping software (PGS) to assist experts in quantifying the weight of evidence. A common challenge lies in quantifying …
View article: #2013 Real-world experience of Iptacopan in C3 glomerulopathy
#2013 Real-world experience of Iptacopan in C3 glomerulopathy Open
Background and Aims C3 Glomerulopathy (C3G) is a rare glomerular disease characterized by dysregulation of the alternative pathway of the complement system. It was relatively recently described, and there remains a need to expand our under…
View article: Integration of structural modeling and CADD scores improve variant classification for CFH SCRs 5-18
Integration of structural modeling and CADD scores improve variant classification for CFH SCRs 5-18 Open
View article: Detecting FH–anti-FH immune complexes in MGRS-C3G
Detecting FH–anti-FH immune complexes in MGRS-C3G Open
View article: Features of circulating factor H immune complexes in complement-mediated diseases
Features of circulating factor H immune complexes in complement-mediated diseases Open
View article: Factor H-related 1 and heparan sulfate architecture contribute to complement dysregulation in C3 glomerulopathy
Factor H-related 1 and heparan sulfate architecture contribute to complement dysregulation in C3 glomerulopathy Open
Introduction Dysregulation of the alternative pathway of complement underlies the pathogenesis of C3 glomerulopathy (C3G). Because Factor H (FH) prevents excessive alternative pathway activity while Factor H-related protein 1 (FHR-1) is be…
View article: Functional and structural characterization of mouse Factor H-related B protein unveils a novel dimerization domain shared by FHR-B and FH
Functional and structural characterization of mouse Factor H-related B protein unveils a novel dimerization domain shared by FHR-B and FH Open
Factor H-related proteins (FHRs) are found in mice, but their equivalence to human FHRs remains uncertain. This study identifies three FHRs in mouse plasma (FHR-B, FHR-C, and FHR-E) and focuses on characterizing FHR-B. Using purified plasm…
View article: Novel immunochromatographic test for rapid detection of anti-factor H autoantibodies with an assessment of its clinical relevance
Novel immunochromatographic test for rapid detection of anti-factor H autoantibodies with an assessment of its clinical relevance Open
Factor H (FH) is a crucial complement regulator that prevents complement-mediated injury to healthy cells and tissues. This regulatory function can be disrupted by Factor H autoantibodies (FHAA), which then leads to diseases such as atypic…
View article: Role of factor H-related protein 3 in Pseudomonas aeruginosa bloodstream infections
Role of factor H-related protein 3 in Pseudomonas aeruginosa bloodstream infections Open
Pseudomonas aeruginosa is a leading cause of nosocomial bloodstream infections. The outcome of these infections depends on the virulence of the microorganism as well as host-related conditions and factors. The complement system plays a cru…
View article: The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference
The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference Open
View article: A Cell-Based Assay to Measure the Activity of the Complement Convertases
A Cell-Based Assay to Measure the Activity of the Complement Convertases Open
View article: WCN24-1027 THE ALTERNATIVE COMPLEMENT PATHWAY IN SHIGA TOXIN-PRODUCING ESCHERICHIA COLI– ASSOCIATED HEMOLYTIC UREMIC SYNDROME (STEC-HUS)
WCN24-1027 THE ALTERNATIVE COMPLEMENT PATHWAY IN SHIGA TOXIN-PRODUCING ESCHERICHIA COLI– ASSOCIATED HEMOLYTIC UREMIC SYNDROME (STEC-HUS) Open
Excessive complement activation plays a crucial role in the pathogenesis of atypical hemolytic uremic syndrome (aHUS). However, its involvement in Shiga Toxin-Producing Escherichia coli– Associated Hemolytic Uremic Syndrome (STEC-HUS) rema…
View article: Factor H-related protein 1 promotes complement-mediated opsonization of Pseudomonas aeruginosa
Factor H-related protein 1 promotes complement-mediated opsonization of Pseudomonas aeruginosa Open
Pseudomonas aeruginosa is an important human opportunistic pathogen responsible for a wide range of infections. The complement system is the main early host defense mechanism to control these infections. P. aeruginosa counteracts complemen…
View article: C4BP(β-)-mediated immunomodulation attenuates inflammation in DSS-induced murine colitis and in myeloid cells from IBD patients
C4BP(β-)-mediated immunomodulation attenuates inflammation in DSS-induced murine colitis and in myeloid cells from IBD patients Open
The most recent and promising therapeutic strategies for inflammatory bowel disease (IBD) have engaged biologics targeting single effector components involved in major steps of the immune-inflammatory processes, such as tumor necrosis fact…
View article: On the relevance of thrombomodulin variants in atypical hemolytic uremic syndrome
On the relevance of thrombomodulin variants in atypical hemolytic uremic syndrome Open
View article: The structure of Leptospira interrogans GAPDH sheds light into an immunoevasion factor that can target the anaphylatoxin C5a of innate immunity
The structure of Leptospira interrogans GAPDH sheds light into an immunoevasion factor that can target the anaphylatoxin C5a of innate immunity Open
Leptospirosis is a neglected worldwide zoonosis involving farm animals and domestic pets caused by the Gram-negative spirochete Leptospira interrogans . This bacterium deploys a variety of immune evasive mechanisms, some of them targeted a…
View article: Clinical Profiles and Patterns of Kidney Disease Progression in C3 Glomerulopathy
Clinical Profiles and Patterns of Kidney Disease Progression in C3 Glomerulopathy Open
Key Points Kidney survival in C3 glomerulopathy is significantly higher in patients with a disease chronicity score <4 and proteinuria <3.5 g/d, regardless of baseline eGFR. A faster eGFR decline in C3 glomerulopathy is associated with hig…
View article: Successful pharmacological intervention at different levels of the complement system in an in vitro complement fixation model for bullous pemphigoid
Successful pharmacological intervention at different levels of the complement system in an in vitro complement fixation model for bullous pemphigoid Open
Bullous pemphigoid (BP) is characterized by deposition of immunoglobulins and complement along the epidermal basement membrane (BM). In humans, there is a lack of functional studies targeting the complement system (CS). This study investig…
View article: Substitutions at position 263 within the von Willebrand factor type A domain determine the functionality of complement C2 protein
Substitutions at position 263 within the von Willebrand factor type A domain determine the functionality of complement C2 protein Open
The complement system is one of the first defense lines protecting from invading pathogens. However, it may turn offensive to the body’s own cells and tissues when deregulated by the presence of rare genetic variants that impair physiologi…
View article: Genetic variability shapes the alternative pathway complement activity and predisposition to complement‐related diseases
Genetic variability shapes the alternative pathway complement activity and predisposition to complement‐related diseases Open
Summary The implementation of next‐generation sequencing technologies has provided a sharp picture of the genetic variability in the components and regulators of the alternative pathway (AP) of the complement system and has revealed the as…
View article: Complement C5 plasma levels are associated to abdominal aortic aneurysm prevalence and progression
Complement C5 plasma levels are associated to abdominal aortic aneurysm prevalence and progression Open
Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Complemento II-CM La Caixa Aim Previous human and experimental studies have suggested a role of complement activation in abdo…
View article: Factor H–Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy
Factor H–Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy Open
Significance Statement Mutations in factor H–related protein 1 (FHR-1) that result in duplication of its dimerization domain associate with the chronic renal disease C3 glomerulopathy (C3G), which is characterized by complement dysregulati…
View article: Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy
Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy Open
Background C3 glomerulopathy is a rare and heterogeneous complement-driven disease. It is often challenging to accurately predict in clinical practice the individual kidney prognosis at baseline. We herein sought to develop and validate a …
View article: The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity
The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity Open
C4b-binding protein (C4BP) is a well-known regulator of the complement system that holds additional and important activities unrelated to complement inhibition. Recently, we have described a novel immunomodulatory activity in the minor C4B…
View article: The crystal structure of iC3b-CR3 αI reveals a modular recognition of the main opsonin iC3b by the CR3 integrin receptor
The crystal structure of iC3b-CR3 αI reveals a modular recognition of the main opsonin iC3b by the CR3 integrin receptor Open
View article: Gain-of-Function Mutations R249C and S250C in Complement C2 Protein Increase C3 Deposition in the Presence of C-Reactive Protein
Gain-of-Function Mutations R249C and S250C in Complement C2 Protein Increase C3 Deposition in the Presence of C-Reactive Protein Open
The impairment of the alternative complement pathway contributes to rare kidney diseases such as atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). We recently described an aHUS patient carrying an exceptional gain-of-f…
View article: Case Report: Combined Liver-Kidney Transplantation to Correct a Mutation in Complement Factor B in an Atypical Hemolytic Uremic Syndrome Patient
Case Report: Combined Liver-Kidney Transplantation to Correct a Mutation in Complement Factor B in an Atypical Hemolytic Uremic Syndrome Patient Open
Pathogenic gain-of-function variants in complement Factor B were identified as causative of atypical Hemolytic Uremic syndrome (aHUS) in 2007. These mutations generate a reduction on the plasma levels of complement C3. A four-month-old boy…
View article: Functional characterization of 105 factor H variants associated with aHUS: lessons for variant classification
Functional characterization of 105 factor H variants associated with aHUS: lessons for variant classification Open
Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy that can progress, when untreated, to end-stage renal disease. Most frequently, aHUS is caused by complement dysregulation due to pathogenic variant…
View article: Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome)
Complement Factor D (adipsin) Levels Are Elevated in Acquired Partial Lipodystrophy (Barraquer–Simons syndrome) Open
Complement overactivation has been reported in most patients with Barraquer–Simons syndrome (BSS), a rare form of acquired partial lipodystrophy. Complement Factor D (FD) is a serine protease with a crucial role in the activation of the al…