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Related Experiment Video

Updated: Jun 11, 2026

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment
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Familial C4B deficiency and immune complex glomerulonephritis.

K Soto1, Y L Wu, A Ortiz

  • 1Department of Nephrology, Hospital Fernando Fonseca, Lisbon, Portugal. ksoto.nefro@gmail.com

Clinical Immunology (Orlando, Fla.)
|June 29, 2010
PubMed
Summary

Complete C4B deficiency was identified in a patient with Membranoproliferative Glomerulonephritis (MPGN) type III, challenging known complement roles. This highlights the importance of genetic and functional complement testing in glomerulopathy patients.

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Published on: June 8, 2022

Area of Science:

  • Immunology
  • Genetics
  • Nephrology

Background:

  • Complement C4B deficiency is rare and its association with glomerulonephritis is not fully understood.
  • Membranoproliferative Glomerulonephritis (MPGN) type III involves immune deposits in the kidneys.
  • The classical complement pathway plays a role in immune response and tissue homeostasis.

Observation:

  • A young female patient presented with Membranoproliferative Glomerulonephritis type III, exhibiting significant C4 and IgG deposits in renal biopsies.
  • Genetic analysis revealed homozygous C4B deficiency due to a homozygous monomodular RCCX genotype with single long C4A genes.
  • Low C4 levels were observed in other family members, some with heterozygous C4B deficiency, but without renal disease or infections.

Findings:

  • The patient's homozygous C4B deficiency was directly linked to her MPGN type III diagnosis.
  • HLA typing showed specific homozygous alleles (A*02, Cw*06, B*50, DRB1*08, DQB1*03) in the patient.
  • The study identified a complete C4B deficiency in the patient and heterozygous deficiency in relatives, challenging established roles of C4A and C4B.

Implications:

  • These findings necessitate a re-evaluation of the pathophysiological roles of C4A and C4B in immune-mediated diseases.
  • Functional assays, C4 allotyping, and genotyping are crucial for diagnosing and understanding glomerulopathies associated with low complement levels.
  • This case underscores the importance of comprehensive genetic and functional complement analysis in patients with unexplained glomerulonephritis and immune deposits.