Related Experiment Video

Updated: May 17, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

C3 glomerulonephritis and CFHR5 nephropathy

Daniel P Gale1, Patrick H Maxwell

  • 1Centre for Nephrology, Division of Medicine, University College London, London, UK. d.gale@ucl.ac.uk

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association
|November 6, 2012
PubMed

Insights

Complement dysregulation causes kidney disease like C3 glomerulonephritis (C3GN). Genetic studies reveal key proteins involved, offering potential therapeutic targets for C3GN and other kidney disorders.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Complement system plays a crucial role in host defense against infection.
  • Dysregulation of complement activation and control can lead to kidney diseases, notably C3 glomerulonephritis (C3GN).
  • C3GN involves abnormal deposition of complement proteins (without immunoglobulins) in the kidney glomeruli.

Purpose of the Study:

  • To elucidate the molecular causes of C3 glomerulonephritis (C3GN).
  • To identify key proteins involved in protecting the kidney from complement-mediated damage.
  • To explore the therapeutic potential of manipulating the complement pathway for kidney diseases.

Main Methods:

  • Studies of single cases and families to gather genetic evidence.
  • Analysis of complement regulatory proteins.
  • Investigation of the genetic basis of C3GN and related glomerular disorders.

Main Results:

  • Identified complement factor H-related 5 (CFHR5) nephropathy as the most frequent cause of C3GN, particularly in Greek Cypriots.
  • Genetic evidence links complement regulators to the development of common glomerular diseases like IgA nephropathy and lupus nephritis.
  • Established the feasibility of therapeutic interventions targeting the complement pathway.

Conclusions:

  • Disordered complement regulation is a significant factor in kidney disease pathogenesis.
  • Understanding the genetic underpinnings of C3GN provides insights into broader glomerular disorders.
  • Targeting the complement pathway presents a promising therapeutic strategy for C3GN and potentially other kidney diseases.

More Related Videos

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment07:26

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

Published on: July 18, 2017

SorLA and CLC:CLF-1-dependent Downregulation of CNTFRα as Demonstrated by Western Blotting, Inhibition of Lysosomal Enzymes, and Immunocytochemistry10:16

SorLA and CLC:CLF-1-dependent Downregulation of CNTFRα as Demonstrated by Western Blotting, Inhibition of Lysosomal Enzymes, and Immunocytochemistry

Published on: January 6, 2017

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cytomegalovirus Disease01:27

Cytomegalovirus Disease

Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
Renal Corpuscle01:20

Renal Corpuscle

The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...