fibronectin glomerulopathy
Findings
No curated finding names fibronectin glomerulopathy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A hereditary kidney disease characterized by proteinuria, type IV renal tubular acidosis, microscopic hematuria and hypertension that may lead to end-stage renal failure in the second to sixth decade of life.
Definition from the Mondo Disease Ontology (MONDO:0007671), read 2026-09-29. CC BY 4.0.
Features
10 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.
- Abnormal glomerular mesangium morphologyHPOHP:0001966
- Very frequent (80% to 99% of cases)
- GlomerulopathyHPOHP:0100820
- Very frequent (80% to 99% of cases)
- HypertensionHPOHP:0000822
- Very frequent (80% to 99% of cases)
- HypoalbuminemiaHPOHP:0003073
- Very frequent (80% to 99% of cases)
- Microscopic hematuriaHPOHP:0002907
- Very frequent (80% to 99% of cases)
- Nephrotic syndromeHPOHP:0000100
- Very frequent (80% to 99% of cases)
- Pedal edemaHPOHP:0010741
- Very frequent (80% to 99% of cases)
- ProteinuriaHPOHP:0000093
- Very frequent (80% to 99% of cases)
- Renal insufficiencyHPOHP:0000083
- Very frequent (80% to 99% of cases)
- Cerebral hemorrhageHPOHP:0001342
- Occasional (5% to 29% 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.
- FN1HGNC:3778
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
2 names
Resolves to: fibronectin glomerulopathy
- Also called
- GFNDglomerulopathy with fibronectin deposits