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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.
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.
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.
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.
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles.
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).
First described 1991. Earliest sequence paper UniProt cites for the protein: Lee et al, Nature, 1991, "Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes". Source.
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)
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).
Query 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.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.
Shares Neuroblastoma (paediatric), IntOGen.