FUS RNA binding protein
Dernière mise à jour 18 Aug 2020
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La FUS gene provides instructions for making a protein that is found within the cell nucleus in most tissues and is involved in many of the steps of protein production.
Normal function
La FUS gene provides instructions for making a protein that is found within the cell nucleus in most tissues and is involved in many of the steps of protein production.
The FUS protein attaches (binds) to DNA and regulates an activity called transcription, which is the first step in the production of proteins from genes. The FUS protein is also involved in processing molecules called messenger RNA (mRNA), which serve as the genetic blueprints for making proteins. By cutting and rearranging mRNA molecules in different ways, the FUS protein controls the production of different versions of certain proteins. This process is known as alternative splicing. Once the FUS protein processes the mRNA, it transports the mRNA out of the nucleus where it gets taken up by other cell structures to be further processed into a mature protein. The FUS protein also helps repair errors in DNA, which prevents cells from accumulating genetic damage.
Conditions referring to this gene or chromosome
Other names
ALS6
ETM4
FUS1
FUS_HUMAN
fused in sarcoma
heterogeneous nuclear ribonucleoprotein P2
hnRNP-P2
HNRNPP2
oncogene FUS
oncogene TLS
POMP75
RNA-binding protein FUS
TLS
translocated in liposarcoma protein
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Lectures complémentaires et références
- FUS RNA binding protein; MedlinePlus, National Library of Medicine.
- Coady TH, Manley JL. ALS mutations in TLS/FUS disrupt target gene expression. Genes Dev. 2015 Aug 15;29(16):1696-706. doi: 10.1101/gad.267286.115. Epub 2015 Aug 6.
- Crozat A, Aman P, Mandahl N, Ron D. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature. 1993 Jun 17;363(6430):640-4. doi: 10.1038/363640a0.
- Deng H, Gao K, Jankovic J. The role of FUS gene variants in neurodegenerative diseases. Nat Rev Neurol. 2014 Jun;10(6):337-48. doi: 10.1038/nrneurol.2014.78. Epub 2014 May 20.
- Hewitt C, Kirby J, Highley JR, Hartley JA, Hibberd R, Hollinger HC, Williams TL, Ince PG, McDermott CJ, Shaw PJ. Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. Arch Neurol. 2010 Apr;67(4):455-61. doi: 10.1001/archneurol.2010.52.
- Panagopoulos I, Storlazzi CT, Fletcher CD, Fletcher JA, Nascimento A, Domanski HA, Wejde J, Brosjo O, Rydholm A, Isaksson M, Mandahl N, Mertens F. The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma. Genes Chromosomes Cancer. 2004 Jul;40(3):218-28. doi: 10.1002/gcc.20037.
- Schwartz JC, Podell ER, Han SS, Berry JD, Eggan KC, Cech TR. FUS is sequestered in nuclear aggregates in ALS patient fibroblasts. Mol Biol Cell. 2014 Sep 1;25(17):2571-8. doi: 10.1091/mbc.E14-05-1007. Epub 2014 Jul 9.
- Storlazzi CT, Mertens F, Nascimento A, Isaksson M, Wejde J, Brosjo O, Mandahl N, Panagopoulos I. Fusion of the FUS and BBF2H7 genes in low grade fibromyxoid sarcoma. Hum Mol Genet. 2003 Sep 15;12(18):2349-58. doi: 10.1093/hmg/ddg237. Epub 2003 Jul 22.
- Vance C, Rogelj B, Hortobagyi T, De Vos KJ, Nishimura AL, Sreedharan J, Hu X, Smith B, Ruddy D, Wright P, Ganesalingam J, Williams KL, Tripathi V, Al-Saraj S, Al-Chalabi A, Leigh PN, Blair IP, Nicholson G, de Belleroche J, Gallo JM, Miller CC, Shaw CE. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science. 2009 Feb 27;323(5918):1208-1211. doi: 10.1126/science.1165942.
- Wang X, Arai S, Song X, Reichart D, Du K, Pascual G, Tempst P, Rosenfeld MG, Glass CK, Kurokawa R. Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription. Nature. 2008 Jul 3;454(7200):126-30. doi: 10.1038/nature06992. Epub 2008 May 28.
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