C3 glomerulonephritis
Findings
No curated finding names C3 glomerulonephritis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Glomerulonephritis characterized by C3 accumulation with little or absent deposition of immunoglobulin, in the absence of ultrastructural electron-dense transformation seen in dense deposit disease.
Definition from the Mondo Disease Ontology (MONDO:0013892), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Progressive
HPO, annotations 2026-09-02
Features
9 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Glomerular C3 depositionHPOHP:0012576
- 9 of 9 reported patients
- Glomerular subendothelial electron-dense depositsHPOHP:0004746
- 9 of 9 reported patients
- Microscopic hematuriaHPOHP:0002907
- 22 of 22 reported patients
- Mesangial hypercellularityHPOHP:0012574
- 8 of 9 reported patients
- Mesangial matrix expansionHPOHP:0033493
- 8 of 9 reported patients
- Thickening of glomerular capillary wallHPOHP:0025005
- 8 of 9 reported patients
- Renal insufficiency
Genes
5 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- CFHHGNC:4883
- Definitive · ClinGen · Autosomal recessive · 2024
- CFHR5HGNC:24668
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2022
- Moderate · Ambry Genetics · Autosomal dominant · 2022
- C3HGNC:1318
- Moderate · ClinGen · Autosomal dominant · 2024
- CFIHGNC:5394
- Moderate · ClinGen · Autosomal dominant · 2024
- CFBHGNC:1037
- Limited · ClinGen · Autosomal dominant · 2024
Where it sits
Other names
2 names
Resolves to: C3 glomerulonephritis
- Also called
- complement-mediated membranoproliferative glomerulonephritisnephropathy due to CFHR5 deficiency