FBN2
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.
Overview
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.
- Target · the protein and the cell it sits on
- Drug · antibody, small molecule, cell or radioligand
- Effect · signal, damage or kill
In plain words · 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.
- 1 · What it is
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.
- 2 · What goes wrong in cancer
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles.
- 3 · How drugs use it
No product in this corpus aims at FBN2 yet. Because the protein is lost rather than overactive, drugs either restore its function or exploit the weakness its loss leaves (synthetic lethality).
External identifiers
Sources: HGNC HGNC:3604 (approved symbol, name, aliases, locus and cross-references (hgnc_complete_set.txt)); UniProt P35556 (protein name, function text, keywords and locations (REST API)); IntOGen FBN2 (driver in 1 cohort (Act 0, LoF 1); Compendium_Cancer_Genes.tsv release 20240920, CC0 1.0)
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).
- Neuroblastoma: IntOGen driver in 1 cohort (NBL)
Notes
top- 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.
Latest papers
topQuery for this target: (TITLE:"FBN2" OR ABSTRACT:"FBN2" OR TITLE:"fibrillin 2" OR ABSTRACT:"fibrillin 2" OR TITLE:"Fibrillin-2" OR ABSTRACT:"Fibrillin-2" OR TITLE:"DA9" OR ABSTRACT:"DA9") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about FBN2, not a curated reading list.
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