# FBN2

Source: https://onco.cc/targets/fbn2/  
OnCo record `fbn2` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

FBN2 (Fibrillin-2) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a tumour suppressor, and it is called a cancer driver by mutation analysis of patient cohorts. Tied to Neuroblastoma.

## Summary

Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fibre assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.

IntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Neuroblastoma.

## Fields

- Kind: Target
- Last checked: 2026-09-23
- Also known as: fibrillin 2; Fibrillin-2; DA9
- Tags: cancer-genes-wave
- Symbol: FBN2
- Class: tumor-suppressor
- Biology: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fibre assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Hormone secreted by trophoblasts that promotes trophoblast invasiveness. Has glucogenic activity: is able to increase plasma glucose levels. Location: Secreted; Secreted, extracellular space, extracellular matrix (UniProt). Locus 5q23.3 (HGNC).
- Where found: Neuroblastoma: IntOGen driver in 1 cohort (NBL)

## Notes

- Written by scripts/fetch-cancer-genes.ts from CIViC, Open Targets, IntOGen, HGNC and UniProt; the function text is UniProt's, condensed and in UK spelling. Roles: IntOGen calls it a loss-of-function (LoF) driver in 1 cohort. Evidence tier "cohort-driver" is the strongest of those signals.
- Prevalence not recorded: none of the sources gives a positivity rate.

## Sources

- HGNC HGNC:3604: https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3604
- UniProt P35556: https://www.uniprot.org/uniprotkb/P35556/entry
- NCBI Gene 2201: https://www.ncbi.nlm.nih.gov/gene/2201
- Ensembl ENSG00000138829: https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000138829

## Connected records

- collections: [IntOGen](https://onco.cc/collections/intogen/)
- cancers: [Neuroblastoma (paediatric)](https://onco.cc/cancers/neuroblastoma/)

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JSON: https://onco.cc/api/v1/entities/fbn2.json