immunoglobulin-mediated membranoproliferative glomerulonephritis
Findings
No curated finding names immunoglobulin-mediated membranoproliferative glomerulonephritis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Glomerulonephritis characterized by mesangial proliferation, endocapillary proliferation, and glomerular capillary wall remodeling with immune complex deposits from classical complement pathway activation.
Definition from the Mondo Disease Ontology (MONDO:0014005), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal recessive inheritance
- Onset and course
- Infantile onset · Juvenile onset · Progressive · Childhood onset · Early young adult onset
HPO, annotations 2026-09-02
Features
19 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.
- Membranoproliferative glomerulonephritisHPOHP:0000793
- 9 of 9 reported patients
- ProteinuriaHPOHP:0000093
- 9 of 9 reported patients
- Frequent (30% to 79% of cases)
- Mesangial hypercellularityHPOHP:0012574
- Very frequent (80% to 99% of cases)
- C3 nephritic factor positivityHPOHP:0030888
- Frequent (30% to 79% of cases)
- Decreased circulating complement C3 concentrationHPOHP:0005421
- Frequent (30% to 79% of cases)
Genes
1 gene
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.
- DGKEHGNC:2852
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal recessive · 2022
- Strong · PanelApp Australia · Autosomal recessive · 2025
- Moderate · Ambry Genetics · Autosomal recessive · 2020
Where it sits
Other names
9 names
Resolves to: immunoglobulin-mediated membranoproliferative glomerulonephritis
- Also called
- Ig-mediated membranoproliferative glomerulonephritisIg-mediated MPGNimmune complex mediated membranoproliferative glomerulonephritisimmunoglobulin-mediated MPGNmembranoproliferative glomerulonephritis type Imesangiocapillary glomerulonephritis type 1nephrotic syndrome, type 7nephrotic syndrome, type 7, with membranoproliferative glomerulonephritisNPHS7