UniProt:P35637 FUS

chain
  • chain:1-526
checksum 88C8E263B7905549
comment
  • FUNCTION 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.
crossReference
databaseName UniProt
dbId 55036
description
  • recommendedName: RNA-binding protein FUS alternativeName: 75 kDa DNA-pairing protein alternativeName: Oncogene FUS alternativeName: Oncogene TLS alternativeName: POMp75 alternativeName: Translocated in liposarcoma protein
displayName UniProt:P35637 FUS
geneName
  • FUS
  • TLS
identifier P35637
isSequenceChanged false
keyword
  • 3D-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:9963647] Weiser, Joel, 2025-08-15
moleculeType Protein
name
  • FUS
otherIdentifier
  • 11745190_a_at
  • 1564802_3p_at
  • 1564802_PM_at
  • 1564802_at
  • 1565717_PM_s_at
  • 1565717_s_at
  • 16818207
  • 200959_3p_at
  • 200959_PM_at
  • 200959_at
  • 217370_3p_x_at
  • 217370_PM_x_at
  • 217370_x_at
  • 231108_PM_at
  • 231108_at
  • 2521
  • 3656910
  • 3656912
  • 3656913
  • 3656914
  • 3656915
  • 3656916
  • 3656917
  • 3656918
  • 3656919
  • 3656920
  • 3656921
  • 3656922
  • 3656923
  • 3656924
  • 3656925
  • 3656926
  • 3656927
  • 3656928
  • 3656932
  • 3656933
  • 3656934
  • 3656935
  • 3656936
  • 3656937
  • 3656938
  • 3656939
  • 3656940
  • 3656941
  • 3656942
  • 3656943
  • 3656944
  • 3656945
  • 3656946
  • 3656947
  • 3656948
  • 3656949
  • 3656951
  • 3656952
  • 3656953
  • 3656955
  • 3656956
  • 3656958
  • 3656960
  • 3656962
  • 3656963
  • 39180_at
  • 43501_at
  • 46825_at
  • 63941_at
  • 73061_at
  • 7995069
  • A_14_P116276
  • A_14_P136409
  • A_14_P136436
  • A_14_P139905
  • A_23_P106887
  • GE58786
  • GE646248
  • GE85981
  • GE85986
  • GE88499
  • GO:0003676
  • GO:0003677
  • GO:0003682
  • GO:0003712
  • GO:0003713
  • GO:0003723
  • GO:0003730
  • GO:0005515
  • GO:0005634
  • GO:0005654
  • GO:0005737
  • GO:0006355
  • GO:0006357
  • GO:0008270
  • GO:0008380
  • GO:0010467
  • GO:0042802
  • GO:0043484
  • GO:0045893
  • GO:0046872
  • GO:0048255
  • GO:0051260
  • GO:0060090
  • GO:0065003
  • GO:0098978
  • GO:0098982
  • GO:0099523
  • GO:0099524
  • GO:0140693
  • GO:0140694
  • GO:1905168
  • GO:1990000
  • HMNXSV003036649
  • HMNXSV003041499
  • Hs.107720.0.A1_3p_at
  • Hs.99969.2.S1_3p_a_at
  • Hs.99969.2.S1_3p_s_at
  • Hs.99969.2.S1_3p_x_at
  • Hs.99969.3.S1_3p_at
  • Hs2.382749.1.S1_3p_x_at
  • Hs2.392171.1.S1_3p_s_at
  • ILMN_2306066
  • PH_hs_0022279
  • PH_hs_0031515
  • TC16000372.hg
  • TC16001494.hg
  • TC16001495.hg
  • X71428_at
  • g4826733_3p_at
  • g4826733_3p_s_at
  • g4826733_3p_x_at
  • p6361
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • FUS_HUMAN
  • Q9H4A8
sequenceLength 526
species [Species:48887] Homo sapiens
stId uniprot:P35637
url http://purl.uniprot.org/uniprot/P35637

Referrals

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