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glomerulopathy with fibronectin deposits 2 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

glomerulopathy with fibronectin deposits 2

MONDO:0011165

Also known as:FN1 fibronectin glomerulopathyfibronectin glomerulopathy caused by mutation in FN1glomerulopathy with fibronectin deposits type 2

Overview

Any fibronectin glomerulopathy in which the cause of the disease is a mutation in the FN1 gene.

GeneticsAutosomal dominant · FN1 (chr 2)

ResearchLimited published research (9 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Glomerular deposits, Protein in the urine (proteinuria), Glomerular fibronectin deposits, and Glomerulomegaly; and very common findings: Hypertension. 9 total HPO annotations.

Organ System	Phenotype Count	Example Features
Kidneys and urinary system	8	Stage 5 chronic kidney disease, Nephrotic syndrome, Reduced kidney function (renal insufficiency)

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

FN1 encodes fibronectin 1 (2,477 aa). Fibronectins bind cell surfaces and various compounds including collagen, fibrin, heparin, DNA, and actin. Highest expression in Cells Cultured fibroblasts (20,887 TPM) and Artery Aorta (3,726 TPM).

Glomerulopathy with fibronectin deposits 2 has been associated with mutations in the FN1 gene on chromosome 2.

The FN1 protein participates in FN1(32-1201)-ALK(1022-1620) fusion and FN1(32-1201)-p-7Y-ALK(1022-1620) fusion pathways.

FN1 is classified as a druggable target (Druggable Genome category) with score 8.0.

Diagnosis

Genetic testing for FN1 is available. Testing is considered supportive for diagnosis.

Prognosis

Phenotype severity distribution: 4 always present features, 1 very common feature, 3 common features.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for glomerulopathy with fibronectin deposits 2.

9 publications have been identified in PubMed for glomerulopathy with fibronectin deposits 2. Research spans Case Report / Case Series (56%) and Review / Meta-Analysis (44%).

Recent Publications

Wei J (2026). [PMID: 42150947](https://pubmed.ncbi.nlm.nih.gov/42150947/). *Transplant Proc*. [Review / Meta-Analysis]
Chen X (2026). [PMID: 42068364](https://pubmed.ncbi.nlm.nih.gov/42068364/). *Pediatr Nephrol*. [Case Report / Case Series]
Shi B (2026). [PMID: 41642109](https://pubmed.ncbi.nlm.nih.gov/41642109/). *Clin Nephrol*. [Review / Meta-Analysis]
Sannino A (2026). [PMID: 42143752](https://pubmed.ncbi.nlm.nih.gov/42143752/). *G Ital Nefrol*. [Case Report / Case Series]
Tsighe AZ (2025). [PMID: 40529320](https://pubmed.ncbi.nlm.nih.gov/40529320/). *Case Rep Nephrol Dial*. [Case Report / Case Series]
Kalmár T (2025). [PMID: 39859354](https://pubmed.ncbi.nlm.nih.gov/39859354/). *Int J Mol Sci*. [Case Report / Case Series]

Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 3:00 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:601894

GARD

Genetic and Rare Diseases Info Center

GARD:9914

Questions for Your Care Team

→Should I pursue genetic testing for FN1, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Kisho

glomerulopathy with fibronectin deposits 2

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Latest News & Developments

Successful Control of Late-Onset Nephrotic Syndrome in FN1-Associated Fibronectin Glomerulopathy: A 17-Year Clinical Course.

Concomitant Fibronectin Glomerulopathy and Chronic Active Antibody-mediated Rejection in a Renal Allograft: A Case Report and Literature Review.

glomerulopathy with fibronectin deposits 2

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Latest News & Developments

Successful Control of Late-Onset Nephrotic Syndrome in FN1-Associated Fibronectin Glomerulopathy: A 17-Year Clinical Course.

Concomitant Fibronectin Glomerulopathy and Chronic Active Antibody-mediated Rejection in a Renal Allograft: A Case Report and Literature Review.