{"entity":{"id":"fbn2","kind":"target","name":"FBN2","aka":["fibrillin 2","Fibrillin-2","DA9"],"tldr":"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.\n\nIntOGen calls it a driver in 1 cohort (0 activating, 1 loss-of-function), covering Neuroblastoma.","asOf":"2026-09-23","links":[{"label":"HGNC HGNC:3604","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3604"},{"label":"UniProt P35556","url":"https://www.uniprot.org/uniprotkb/P35556/entry"},{"label":"NCBI Gene 2201","url":"https://www.ncbi.nlm.nih.gov/gene/2201"},{"label":"Ensembl ENSG00000138829","url":"https://www.ensembl.org/Homo_sapiens/Gene/Summary?g=ENSG00000138829"}],"tags":["cancer-genes-wave"],"related":["intogen"],"cancers":["neuroblastoma"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"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."],"provenance":{"editedBy":"scripts/fetch-cancer-genes.ts (CIViC, Open Targets, IntOGen, HGNC, UniProt)","editedOn":"2026-09-23"},"symbol":"FBN2","role":["tumour-suppressor"],"evidenceTier":"cohort-driver","sources":[{"label":"HGNC HGNC:3604","url":"https://www.genenames.org/data/gene-symbol-report/#!/hgnc_id/HGNC:3604","note":"approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)"},{"label":"UniProt P35556","url":"https://www.uniprot.org/uniprotkb/P35556/entry","note":"protein name, function text, keywords and locations (REST API)"},{"label":"IntOGen FBN2","url":"https://www.intogen.org/search?gene=FBN2","note":"driver in 1 cohort (Act 0, LoF 1); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0"}],"hgnc":"HGNC:3604","ensembl":"ENSG00000138829","uniprot":"P35556","entrez":"2201","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).","whereFound":["Neuroblastoma: IntOGen driver in 1 cohort (NBL)"],"targetClass":"tumor-suppressor","prevalence":[]},"route":"/targets/fbn2/","neighbours":{"collection":[{"id":"intogen","kind":"collection","name":"IntOGen","route":"/collections/intogen/"}],"cancer":[{"id":"neuroblastoma","kind":"cancer","name":"Neuroblastoma (paediatric)","route":"/cancers/neuroblastoma/"}]}}