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Details on Person UniProt:P35637 FUS

Class:IdReferenceGeneProduct:55036
_chainChangeLogchain:1-526 added on Fri February 6 2015
_displayNameUniProt:P35637 FUS
_timestamp2025-08-15 21:22:39
chainchain:1-526
checksum88C8E263B7905549
commentFUNCTION DNA/RNA-binding protein that plays a role in various cellular processes such as transcription regulation, RNA splicing, RNA transport, DNA repair and damage response (PubMed:27731383). Binds to ssRNA containing the consensus sequence 5'-AGGUAA-3' (PubMed:21256132). Binds to nascent pre-mRNAs and acts as a molecular mediator between RNA polymerase II and U1 small nuclear ribonucleoprotein thereby coupling transcription and splicing (PubMed:26124092). Also binds its own pre-mRNA and autoregulates its expression; this autoregulation mechanism is mediated by non-sense-mediated decay (PubMed:24204307). Plays a role in DNA repair mechanisms by promoting D-loop formation and homologous recombination during DNA double-strand break repair (PubMed:10567410). In neuronal cells, plays crucial roles in dendritic spine formation and stability, RNA transport, mRNA stability and synaptic homeostasis (By similarity).SUBUNIT Self-oligomerizes (via N-terminal region) (PubMed:25453086). Oligomerization is essential for chromatin binding (PubMed:25453086). Component of nuclear riboprotein complexes. Interacts with ILF3, TDRD3 and SF1 (PubMed:9660765). Interacts through its C-terminus with SFRS13A (PubMed:9774382). Interacts with OTUB1 and SARNP. Interacts with LRSAM1 (PubMed:27615052). Interacts with SAFB1 in a DNA-dependent manner; this interaction tethers FUS to chromatin (PubMed:27731383). Interacts with MATR3 (PubMed:27731383). Interacts with SNRNP70 and POLR2A; these interactions couple RNA transcription and splicing (PubMed:26124092). Interacts (through its RNA-binding domain) with RALY (through its RNA-binding domain); both are components of the same RNPs (PubMed:30354839).INTERACTION Displays a punctate pattern inside the nucleus and is excluded from nucleoli.ALTERNATIVE PRODUCTS Ubiquitous.PTM Arg-216 and Arg-218 are dimethylated, probably to asymmetric dimethylarginine.PTM Phosphorylated in its N-terminal serine residues upon induced DNA damage. ATM and DNA-PK are able to phosphorylate FUS N-terminal region.DISEASE A chromosomal aberration involving FUS is found in a patient with malignant myxoid liposarcoma. Translocation t(12;16)(q13;p11) with DDIT3.DISEASE A chromosomal aberration involving FUS is a cause of acute myeloid leukemia (AML). Translocation t(16;21)(p11;q22) with ERG.DISEASE The gene represented in this entry is involved in disease pathogenesis. A chromosomal aberration involving FUS is found in a patient with angiomatoid fibrous histiocytoma. Translocation t(12;16)(q13;p11.2) with ATF1 generates a chimeric FUS/ATF1 protein.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the RRM TET family.
descriptionrecommendedName: RNA-binding protein FUS alternativeName: 75 kDa DNA-pairing protein alternativeName: Oncogene FUS alternativeName: Oncogene TLS alternativeName: POMp75 alternativeName: Translocated in liposarcoma protein
geneNameFUS
TLS
identifierP35637
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Amyotrophic lateral sclerosis
Chromosomal rearrangement
Direct protein sequencing
Disease variant
DNA-binding
Isopeptide bond
Metal-binding
Methylation
Neurodegeneration
Nucleus
Phosphoprotein
Proteomics identification
Proto-oncogene
Reference proteome
Repeat
RNA-binding
Ubl conjugation
Zinc
Zinc-finger
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9948485] Weiser, Joel, 2025-05-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameFUS
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998316] ENSEMBL:ENSG00000089280 FUS [Homo sapiens]
secondaryIdentifierFUS_HUMAN
Q9H4A8
sequenceLength526
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:147483] UniProt:P35637-2 FUS [Homo sapiens]
[ReferenceIsoform:403841] UniProt:P35637-1 FUS [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:72040] FUS [nucleoplasm] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P35637 FUS (55036)