Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
Complement System01:27

Complement System

The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a membrane...
Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...
Extrinsic and Intrinsic Pathways of Hemostasis01:20

Extrinsic and Intrinsic Pathways of Hemostasis

Blood clotting or coagulation involves extrinsic and intrinsic pathways, which ultimately merge into the common pathway, forming a fibrin clot.
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which forms a...
Humoral Immune Responses01:36

Humoral Immune Responses

Overview
Disorders of Hemostasis01:24

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

ApoE- and Cfh-deficient mice exhibit structural and molecular features of human early-intermediate retinal degeneration.

Animal models and experimental medicine·2026
Same author

Intravitreal faricimab pharmacokinetics assessed by PET imaging in a neovascular Age-related Macular Degeneration rat model.

International journal of pharmaceutics: X·2026
Same author

Beyond monotherapy in diabetic macular edema: sequential and combination therapy-when and why?

Archivos de la Sociedad Espanola de Oftalmologia·2026
Same author

The authors reply.

Kidney international·2026
Same author

Combined versus Sequential Surgery in Lamellar Macular Holes: A Multicenter Observational Study.

Clinical ophthalmology (Auckland, N.Z.)·2026
Same author

Vascular endothelial growth factor inhibitors outcomes in good vision eyes with neovascular age-related macular degeneration: Fight Retinal Blindness! SPAIN Report 4.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie·2026

Related Experiment Video

Updated: Jul 14, 2026

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
06:29

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells

Published on: January 29, 2014

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

Matthew C Pickering1, Elena Goicoechea de Jorge, Rubén Martinez-Barricarte

  • 1Molecular Genetics and Rheumatology Section, Faculty of Medicine, Imperial College, London W12 0NN, England, UK. matthew.pickering@imperial.ac.uk

The Journal of Experimental Medicine
|May 23, 2007
PubMed
Summary

Factor H (FH) regulates complement activation. Mutations in the FH gene cause diseases like aHUS, MPGN2, and AMD, with distinct genetic links and pathologies, as shown in a novel mouse model.

More Related Videos

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment
07:26

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

Measuring Erythrocyte Complement Receptor 1 Using Flow Cytometry
07:20

Measuring Erythrocyte Complement Receptor 1 Using Flow Cytometry

Published on: May 19, 2020

Related Experiment Videos

Last Updated: Jul 14, 2026

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
06:29

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells

Published on: January 29, 2014

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment
07:26

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

Measuring Erythrocyte Complement Receptor 1 Using Flow Cytometry
07:20

Measuring Erythrocyte Complement Receptor 1 Using Flow Cytometry

Published on: May 19, 2020

Area of Science:

  • Immunology
  • Genetics
  • Nephrology

Background:

  • Factor H (FH) is a key regulator of the complement system's alternative pathway.
  • Polymorphisms in the FH gene are linked to Age-related Macular Degeneration (AMD), atypical Hemolytic Uremic Syndrome (aHUS), and Membranoproliferative Glomerulonephritis type II (MPGN2).
  • These diseases exhibit distinct pathological features, including complement deposition in the eye/kidney (AMD/MPGN2) or renal endothelial injury (aHUS).

Purpose of the Study:

  • To investigate the genotype-phenotype relationship between FH gene variations and associated diseases.
  • To develop and utilize a mouse model to study the in vivo pathogenesis of FH-associated disorders.
  • To elucidate the specific roles of FH in complement regulation and disease development.

Main Methods:

  • Cfh haplotype association analysis in patients with AMD, aHUS, and MPGN2.
  • Generation and characterization of FH-deficient mice.
  • Transgenic expression of aHUS-associated human FH mutants in FH-deficient mice.

Main Results:

  • Distinct Cfh risk haplotypes were identified for AMD/MPGN2 versus aHUS.
  • FH-deficient mice developed MPGN2 but not aHUS.
  • Transgenic mice expressing aHUS-associated FH mutants developed aHUS but not MPGN2, demonstrating effective plasma C3 regulation but defective endothelial complement control.

Conclusions:

  • The study establishes a clear genotype-phenotype correlation for FH-associated diseases.
  • A novel mouse model for aHUS was developed, providing in vivo evidence for its pathogenesis.
  • Defective complement control on renal endothelium, alongside effective plasma C3 regulation, is critical in FH-associated aHUS.