RNA-binding protein FUS (FUsed in Sarcoma) is a protein

Protein

Proteins are biochemical compounds consisting of one or more polypeptides typically folded into a globular or fibrous form, facilitating a biological function. A polypeptide is a single linear polymer chain of amino acids bonded together by peptide bonds between the carboxyl and amino groups of...

 that in humans is encoded by the FUS gene

Gene

A gene is a molecular unit of heredity of a living organism. It is a name given to some stretches of DNA and RNA that code for a type of protein or for an RNA chain that has a function in the organism. Living beings depend on genes, as they specify all proteins and functional RNA chains...

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Function

The N-terminal end of FUS appears to be involved in transcriptional activation, while the C-terminal end is involved in protein and RNA binding. In addition recognition sites for the transcription factors AP2

Activating protein 2

Activating Protein 2 is a family of closely related transcription factors which plays a critical role in regulating gene expression during early development....

, GCF

C2orf3

GC-rich sequence DNA-binding factor is a protein that in humans is encoded by the C2orf3 gene.-Further reading:...

, Sp1 have been identified in FUS.

Clinical significance

FUS has recently been implicated as causative for approximately 1 in 20 cases of familial (inherited) amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis , also referred to as Lou Gehrig's disease, is a form of motor neuron disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input...

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Furthermore, FUS positive cytoplasmic and intranuclear inclusion bodies have been identified in the Tau negative-Ubiquitin positive-TDP 43 negative subtype of Frontotemporal Dementia. This subtype is now referred to as FTLD-FUS. Thus far it has been found that in a similar fashion to the FTLD-TDP43 subtype, the FUS protein aggregating in these inclusions are free of any pathogenic mutations. This contrasts with ALS, in which several causative mutations in FUS have been identified.

FUS gene rearrangement has been implicated in the pathogenesis of both myxoid liposarcoma and low grade fibromyxoid sarcoma.

Interactions

FUS has been shown to interact

Protein-protein interaction

Protein–protein interactions occur when two or more proteins bind together, often to carry out their biological function. Many of the most important molecular processes in the cell such as DNA replication are carried out by large molecular machines that are built from a large number of protein...

 with ILF3

ILF3

Interleukin enhancer-binding factor 3 is a protein that in humans is encoded by the ILF3 gene.-Interactions:ILF3 has been shown to interact with XPO5, FUS, Protein kinase R, PRMT1 and DNA-PKcs....

, RELA

RELA

Transcription factor p65 is a protein that in humans is encoded by the RELA gene.-Interactions:RELA has been shown to interact with NFKBIB, ETHE1, NFKBIE, RFC1, TRIB3, CREB binding protein, Neutrophil cytosolic factor 1, Glucocorticoid receptor, MTPN, BRCA1, C-Fos, POU2F1, BTRC, TATA-binding...

, SPI1

SPI1

Transcription factor PU.1 is a protein that in humans is encoded by the SPI1 gene.-Interactions:SPI1 has been shown to interact with NONO, GATA2, FUS and IRF4.-See also:*Invasion gene associated RNA , a ncRNA located in the SPI-1 region...

, FUSIP1/SRSF10 and PRMT1

PRMT1

Protein arginine N-methyltransferase 1 is an enzyme that in humans is encoded by the PRMT1 gene.-Interactions:PRMT1 has been shown to interact with BTG2, KHDRBS1, ILF3, HNRPK, BTG1, HNRNPR, IFNAR1, FUS, DHX9 and SUPT5H.-Further reading:...

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Further reading