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Protein

RNA-binding protein FUS

Gene

FUS

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Binds both single-stranded and double-stranded DNA and promotes ATP-independent annealing of complementary single-stranded DNAs and D-loop formation in superhelical double-stranded DNA. May play a role in maintenance of genomic integrity.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section specifies the position(s) and type(s) of zinc fingers within the protein.<p><a href='/help/zn_fing' target='_top'>More...</a></p>Zinc fingeri422 – 453RanBP2-typePROSITE-ProRule annotationAdd BLAST32

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionDNA-binding, RNA-binding
LigandMetal-binding, Zinc

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-72163 mRNA Splicing - Major Pathway

SIGNOR Signaling Network Open Resource

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SIGNORi
P35637

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
RNA-binding protein FUS
Alternative name(s):
75 kDa DNA-pairing protein
Oncogene FUS
Oncogene TLS
POMp75
Translocated in liposarcoma protein
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:FUS
Synonyms:TLS
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 16

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000089280.18

Human Gene Nomenclature Database

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HGNCi
HGNC:4010 FUS

Online Mendelian Inheritance in Man (OMIM)

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MIMi
137070 gene

neXtProt; the human protein knowledge platform

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neXtProti
NX_P35637

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Nucleus

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

A chromosomal aberration involving FUS is found in a patient with malignant myxoid liposarcoma. Translocation t(12;16)(q13;p11) with DDIT3.1 Publication
A chromosomal aberration involving FUS is a cause of acute myeloid leukemia (AML). Translocation t(16;21)(p11;q22) with ERG.1 Publication
Angiomatoid fibrous histiocytoma (AFH)1 Publication
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.1 Publication
Disease descriptionA distinct variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is manifest by nodular subcutaneous growth. Characteristic microscopic features include lobulated sheets of histiocyte-like cells intimately associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a lymph node metastasis.
See also OMIM:612160
Amyotrophic lateral sclerosis 6, with or without frontotemporal dementia (ALS6)5 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
See also OMIM:608030
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_068918191G → S in ALS6. 1 PublicationCorresponds to variant dbSNP:rs148758737Ensembl.1
Natural variantiVAR_068920225G → V in ALS6. 1 Publication1
Natural variantiVAR_068921230G → C in ALS6. 1 PublicationCorresponds to variant dbSNP:rs748374535Ensembl.1
Natural variantiVAR_068922234R → C in ALS6. 1 PublicationCorresponds to variant dbSNP:rs777819849Ensembl.1
Natural variantiVAR_054837244R → C in ALS6. 1 Publication1
Natural variantiVAR_068924507G → D in ALS6. 1 PublicationCorresponds to variant dbSNP:rs267606831EnsemblClinVar.1
Natural variantiVAR_054838514R → G in ALS6. 1 Publication1
Natural variantiVAR_054839514R → S in ALS6. 1 Publication1
Natural variantiVAR_054840515G → C in ALS6. 1 Publication1
Natural variantiVAR_054842518R → K in ALS6. 1 PublicationCorresponds to variant dbSNP:rs121909669EnsemblClinVar.1
Natural variantiVAR_054843521R → C in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 3 PublicationsCorresponds to variant dbSNP:rs121909668EnsemblClinVar.1
Natural variantiVAR_054844521R → G in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 1 PublicationCorresponds to variant dbSNP:rs121909668EnsemblClinVar.1
Natural variantiVAR_054845521R → H in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 4 PublicationsCorresponds to variant dbSNP:rs121909671EnsemblClinVar.1
Natural variantiVAR_054846522R → G in ALS6. 1 Publication1
Natural variantiVAR_054847524R → S in ALS6. 1 PublicationCorresponds to variant dbSNP:rs886041389EnsemblClinVar.1
Natural variantiVAR_054848524R → T in ALS6. 1 Publication1
Natural variantiVAR_054849525P → L in ALS6. 1 PublicationCorresponds to variant dbSNP:rs886041390EnsemblClinVar.1
Natural variantiVAR_077328526Y → YY in ALS6; unknown pathological significance. 1 Publication1
Tremor, hereditary essential 4 (ETM4)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA common movement disorder mainly characterized by postural tremor of the arms. Head, legs, trunk, voice, jaw, and facial muscles also may be involved. The condition can be aggravated by emotions, hunger, fatigue and temperature extremes, and may cause a functional disability or even incapacitation. Inheritance is autosomal dominant.
See also OMIM:614782
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_068923431P → L in ETM4. 1 PublicationCorresponds to variant dbSNP:rs186547381EnsemblClinVar.1

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei175Breakpoint for translocation to form chimeric FUS/ATF1 protein1
Sitei266 – 267Breakpoint for translocation to form FUS/TLS-CHOP oncogene2

Keywords - Diseasei

Amyotrophic lateral sclerosis, Disease mutation, Neurodegeneration, Proto-oncogene

Organism-specific databases

DisGeNET

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DisGeNETi
2521

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

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GeneReviewsi
FUS

MalaCards human disease database

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MalaCardsi
FUS
MIMi608030 phenotype
612160 phenotype
614782 phenotype

Open Targets

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OpenTargetsi
ENSG00000089280

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
803 Amyotrophic lateral sclerosis
275872 Frontotemporal dementia with motor neuron disease
300605 Juvenile amyotrophic lateral sclerosis
79105 Myxofibrosarcoma
99967 Myxoid/round cell liposarcoma
862 NON RARE IN EUROPE: Hereditary essential tremor

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA28425

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
FUS

Domain mapping of disease mutations (DMDM)

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DMDMi
544357

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00000815911 – 526RNA-binding protein FUSAdd BLAST526

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei216Asymmetric dimethylarginine; alternateCombined sources1
Modified residuei216Omega-N-methylarginine; alternateCombined sources1
Modified residuei218Asymmetric dimethylarginine; alternateCombined sources1
Modified residuei218Omega-N-methylarginine; alternateCombined sources1
Modified residuei221PhosphoserineCombined sources1
Modified residuei242Asymmetric dimethylarginine1 Publication1
Modified residuei244Asymmetric dimethylarginine1 Publication1
Modified residuei248Asymmetric dimethylarginine1 Publication1
Modified residuei251Asymmetric dimethylarginine1 Publication1
Modified residuei259Asymmetric dimethylarginine1 Publication1
Modified residuei277PhosphoserineCombined sources1
Modified residuei286PhosphothreonineCombined sources1
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section describes <strong>covalent linkages</strong> of various types formed <strong>between two proteins (interchain cross-links)</strong> or <strong>between two parts of the same protein (intrachain cross-links)</strong>, except the disulfide bonds that are annotated in the <a href="http://www.uniprot.org/manual/disulfid">'Disulfide bond'</a> subsection.<p><a href='/help/crosslnk' target='_top'>More...</a></p>Cross-linki334Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei340PhosphoserineCombined sources1
Modified residuei377Asymmetric dimethylarginine1 Publication1
Modified residuei383Asymmetric dimethylarginine1 Publication1
Modified residuei386Asymmetric dimethylarginine1 Publication1
Modified residuei388Asymmetric dimethylarginine1 Publication1
Modified residuei394Asymmetric dimethylarginine1 Publication1
Modified residuei407Asymmetric dimethylarginine; alternate1 Publication1
Modified residuei407Omega-N-methylarginine; alternateBy similarity1
Modified residuei473Asymmetric dimethylarginine1 Publication1
Modified residuei476Asymmetric dimethylarginine1 Publication1
Modified residuei481Asymmetric dimethylarginine1 Publication1
Modified residuei485Asymmetric dimethylarginine1 Publication1
Modified residuei487Asymmetric dimethylarginine1 Publication1
Modified residuei491Asymmetric dimethylarginine1 Publication1
Modified residuei495Asymmetric dimethylarginine1 Publication1
Modified residuei498Asymmetric dimethylarginine1 Publication1
Modified residuei503Asymmetric dimethylarginine; alternate1 Publication1
Modified residuei503Omega-N-methylarginine; alternateCombined sources1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Arg-216 and Arg-218 are dimethylated, probably to asymmetric dimethylarginine.

Keywords - PTMi

Isopeptide bond, Methylation, Phosphoprotein, Ubl conjugation

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P35637

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
P35637

PeptideAtlas

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PeptideAtlasi
P35637

PRoteomics IDEntifications database

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PRIDEi
P35637

ProteomicsDB human proteome resource

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ProteomicsDBi
55119
55120 [P35637-2]

Consortium for Top Down Proteomics

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TopDownProteomicsi
P35637-1 [P35637-1]
P35637-2 [P35637-2]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P35637

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
P35637

SwissPalm database of S-palmitoylation events

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SwissPalmi
P35637

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Ubiquitous.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000089280 Expressed in 238 organ(s), highest expression level in right testis

CleanEx database of gene expression profiles

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CleanExi
HS_FUS

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P35637 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P35637 HS

Organism-specific databases

Human Protein Atlas

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HPAi
CAB033036
CAB058691
HPA008784

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Component of nuclear riboprotein complexes. Interacts with ILF3, TDRD3 and SF1. Interacts through its C-terminus with SFRS13A. Interacts with OTUB1 and SARNP.Interacts with LRSAM1 (PubMed:27615052).5 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
108797, 416 interactors

CORUM comprehensive resource of mammalian protein complexes

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CORUMi
P35637

Database of interacting proteins

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DIPi
DIP-29857N

Protein interaction database and analysis system

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IntActi
P35637, 74 interactors

Molecular INTeraction database

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MINTi
P35637

STRING: functional protein association networks

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STRINGi
9606.ENSP00000254108

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1526
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Database of protein disorder

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DisProti
DP01102

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P35637

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P35637

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini285 – 371RRMPROSITE-ProRule annotationAdd BLAST87

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes the position of regions of compositional bias within the protein and the particular amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi1 – 165Gln/Gly/Ser/Tyr-richAdd BLAST165
Compositional biasi166 – 267Gly-richAdd BLAST102
Compositional biasi371 – 526Arg/Gly-richAdd BLAST156

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the RRM TET family.Curated

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri422 – 453RanBP2-typePROSITE-ProRule annotationAdd BLAST32

Keywords - Domaini

Repeat, Zinc-finger

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
ENOG410IP58 Eukaryota
ENOG4111GEG LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000157290

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000038010

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG095056

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P35637

KEGG Orthology (KO)

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KOi
K13098

Database for complete collections of gene phylogenies

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PhylomeDBi
P35637

TreeFam database of animal gene trees

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TreeFami
TF322599

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
3.30.70.330, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR034459 FUS
IPR012677 Nucleotide-bd_a/b_plait_sf
IPR035979 RBD_domain_sf
IPR000504 RRM_dom
IPR034870 TET_fam
IPR001876 Znf_RanBP2
IPR036443 Znf_RanBP2_sf

The PANTHER Classification System

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PANTHERi
PTHR23238 PTHR23238, 1 hit
PTHR23238:SF5 PTHR23238:SF5, 1 hit

Pfam protein domain database

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Pfami
View protein in Pfam
PF00076 RRM_1, 1 hit
PF00641 zf-RanBP, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00360 RRM, 1 hit
SM00547 ZnF_RBZ, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF54928 SSF54928, 1 hit
SSF90209 SSF90209, 1 hit

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS50102 RRM, 1 hit
PS01358 ZF_RANBP2_1, 1 hit
PS50199 ZF_RANBP2_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform Long (identifier: P35637-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MASNDYTQQA TQSYGAYPTQ PGQGYSQQSS QPYGQQSYSG YSQSTDTSGY
60 70 80 90 100
GQSSYSSYGQ SQNTGYGTQS TPQGYGSTGG YGSSQSSQSS YGQQSSYPGY
110 120 130 140 150
GQQPAPSSTS GSYGSSSQSS SYGQPQSGSY SQQPSYGGQQ QSYGQQQSYN
160 170 180 190 200
PPQGYGQQNQ YNSSSGGGGG GGGGGNYGQD QSSMSSGGGS GGGYGNQDQS
210 220 230 240 250
GGGGSGGYGQ QDRGGRGRGG SGGGGGGGGG GYNRSSGGYE PRGRGGGRGG
260 270 280 290 300
RGGMGGSDRG GFNKFGGPRD QGSRHDSEQD NSDNNTIFVQ GLGENVTIES
310 320 330 340 350
VADYFKQIGI IKTNKKTGQP MINLYTDRET GKLKGEATVS FDDPPSAKAA
360 370 380 390 400
IDWFDGKEFS GNPIKVSFAT RRADFNRGGG NGRGGRGRGG PMGRGGYGGG
410 420 430 440 450
GSGGGGRGGF PSGGGGGGGQ QRAGDWKCPN PTCENMNFSW RNECNQCKAP
460 470 480 490 500
KPDGPGGGPG GSHMGGNYGD DRRGGRGGYD RGGYRGRGGD RGGFRGGRGG
510 520
GDRGGFGPGK MDSRGEHRQD RRERPY
Length:526
Mass (Da):53,426
Last modified:June 1, 1994 - v1
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i88C8E263B7905549
GO
Isoform Short (identifier: P35637-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     64-65: TG → S

Show »
Length:525
Mass (Da):53,355
Checksum:i949558C718B4C19B
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H3BPE7H3BPE7_HUMAN
RNA-binding protein FUS
FUS
527Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H3BNZ4H3BNZ4_HUMAN
RNA-binding protein FUS
FUS
263Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti338T → N AA sequence (PubMed:10567410).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_068918191G → S in ALS6. 1 PublicationCorresponds to variant dbSNP:rs148758737Ensembl.1
Natural variantiVAR_068919216R → C in ALS6 and ETM4. 2 PublicationsCorresponds to variant dbSNP:rs267606832EnsemblClinVar.1
Natural variantiVAR_068920225G → V in ALS6. 1 Publication1
Natural variantiVAR_068921230G → C in ALS6. 1 PublicationCorresponds to variant dbSNP:rs748374535Ensembl.1
Natural variantiVAR_068922234R → C in ALS6. 1 PublicationCorresponds to variant dbSNP:rs777819849Ensembl.1
Natural variantiVAR_054837244R → C in ALS6. 1 Publication1
Natural variantiVAR_065229254M → V Found in a patient with frontotemporal lobar degeneration. 1 PublicationCorresponds to variant dbSNP:rs752076094EnsemblClinVar.1
Natural variantiVAR_035481312K → Q in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_068923431P → L in ETM4. 1 PublicationCorresponds to variant dbSNP:rs186547381EnsemblClinVar.1
Natural variantiVAR_068924507G → D in ALS6. 1 PublicationCorresponds to variant dbSNP:rs267606831EnsemblClinVar.1
Natural variantiVAR_054838514R → G in ALS6. 1 Publication1
Natural variantiVAR_054839514R → S in ALS6. 1 Publication1
Natural variantiVAR_054840515G → C in ALS6. 1 Publication1
Natural variantiVAR_054841517H → Q Does not affect protein nuclear localization. 1 PublicationCorresponds to variant dbSNP:rs121909667EnsemblClinVar.1
Natural variantiVAR_054842518R → K in ALS6. 1 PublicationCorresponds to variant dbSNP:rs121909669EnsemblClinVar.1
Natural variantiVAR_054843521R → C in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 3 PublicationsCorresponds to variant dbSNP:rs121909668EnsemblClinVar.1
Natural variantiVAR_054844521R → G in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 1 PublicationCorresponds to variant dbSNP:rs121909668EnsemblClinVar.1
Natural variantiVAR_054845521R → H in ALS6; results in aberrant trafficking and cytoplasmic retention of the protein. 4 PublicationsCorresponds to variant dbSNP:rs121909671EnsemblClinVar.1
Natural variantiVAR_054846522R → G in ALS6. 1 Publication1
Natural variantiVAR_054847524R → S in ALS6. 1 PublicationCorresponds to variant dbSNP:rs886041389EnsemblClinVar.1
Natural variantiVAR_054848524R → T in ALS6. 1 Publication1
Natural variantiVAR_054849525P → L in ALS6. 1 PublicationCorresponds to variant dbSNP:rs886041390EnsemblClinVar.1
Natural variantiVAR_077328526Y → YY in ALS6; unknown pathological significance. 1 Publication1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_00579864 – 65TG → S in isoform Short. 1 Publication2

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
S62140 mRNA Translation: AAB27102.1
S62138 mRNA Translation: AAB27103.1 Sequence problems.
X71427 mRNA Translation: CAA50558.1 Sequence problems.
X71428 mRNA Translation: CAA50559.1 Sequence problems.
AF071213 Genomic DNA Translation: AAC35285.1
AF071213 Genomic DNA Translation: AAC35284.1
AC009088 Genomic DNA No translation available.
BC000402 mRNA Translation: AAH00402.1
BC002459 mRNA Translation: AAH02459.1
AJ295163 mRNA Translation: CAC15058.1 Different termination.

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS10707.1 [P35637-1]
CCDS58454.1 [P35637-2]

Protein sequence database of the Protein Information Resource

More...
PIRi
S33798
S33799

NCBI Reference Sequences

More...
RefSeqi
NP_001164105.1, NM_001170634.1 [P35637-2]
NP_004951.1, NM_004960.3 [P35637-1]

UniGene gene-oriented nucleotide sequence clusters

More...
UniGenei
Hs.46894

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000254108; ENSP00000254108; ENSG00000089280 [P35637-1]
ENST00000380244; ENSP00000369594; ENSG00000089280 [P35637-2]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
2521

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:2521

UCSC genome browser

More...
UCSCi
uc002ebh.4 human [P35637-1]

Keywords - Coding sequence diversityi

Alternative splicing, Chromosomal rearrangement, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
S62140 mRNA Translation: AAB27102.1
S62138 mRNA Translation: AAB27103.1 Sequence problems.
X71427 mRNA Translation: CAA50558.1 Sequence problems.
X71428 mRNA Translation: CAA50559.1 Sequence problems.
AF071213 Genomic DNA Translation: AAC35285.1
AF071213 Genomic DNA Translation: AAC35284.1
AC009088 Genomic DNA No translation available.
BC000402 mRNA Translation: AAH00402.1
BC002459 mRNA Translation: AAH02459.1
AJ295163 mRNA Translation: CAC15058.1 Different termination.
CCDSiCCDS10707.1 [P35637-1]
CCDS58454.1 [P35637-2]
PIRiS33798
S33799
RefSeqiNP_001164105.1, NM_001170634.1 [P35637-2]
NP_004951.1, NM_004960.3 [P35637-1]
UniGeneiHs.46894

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...
PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

More...
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2LA6NMR-A282-370[»]
2LCWNMR-A278-385[»]
4FDDX-ray2.30B498-526[»]
4FQ3X-ray3.00B495-526[»]
5W3NNMR-A/B/C/D/E/F/G/H/I2-214[»]
5XRRX-ray1.50A54-59[»]
5XSGelectron microscopy0.73A37-42[»]
5YVGX-ray4.05X/Y1-526[»]
5YVHX-ray3.15B371-526[»]
5YVIX-ray2.90B456-526[»]
6BWZX-ray1.10A37-42[»]
6BXVX-ray1.10A54-61[»]
6BZPelectron microscopy1.10A/B77-82[»]
DisProtiDP01102
ProteinModelPortaliP35637
SMRiP35637
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108797, 416 interactors
CORUMiP35637
DIPiDIP-29857N
IntActiP35637, 74 interactors
MINTiP35637
STRINGi9606.ENSP00000254108

PTM databases

iPTMnetiP35637
PhosphoSitePlusiP35637
SwissPalmiP35637

Polymorphism and mutation databases

BioMutaiFUS
DMDMi544357

Proteomic databases

EPDiP35637
PaxDbiP35637
PeptideAtlasiP35637
PRIDEiP35637
ProteomicsDBi55119
55120 [P35637-2]
TopDownProteomicsiP35637-1 [P35637-1]
P35637-2 [P35637-2]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
2521
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000254108; ENSP00000254108; ENSG00000089280 [P35637-1]
ENST00000380244; ENSP00000369594; ENSG00000089280 [P35637-2]
GeneIDi2521
KEGGihsa:2521
UCSCiuc002ebh.4 human [P35637-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
2521
DisGeNETi2521
EuPathDBiHostDB:ENSG00000089280.18

GeneCards: human genes, protein and diseases

More...
GeneCardsi
FUS
GeneReviewsiFUS
HGNCiHGNC:4010 FUS
HPAiCAB033036
CAB058691
HPA008784
MalaCardsiFUS
MIMi137070 gene
608030 phenotype
612160 phenotype
614782 phenotype
neXtProtiNX_P35637
OpenTargetsiENSG00000089280
Orphaneti803 Amyotrophic lateral sclerosis
275872 Frontotemporal dementia with motor neuron disease
300605 Juvenile amyotrophic lateral sclerosis
79105 Myxofibrosarcoma
99967 Myxoid/round cell liposarcoma
862 NON RARE IN EUROPE: Hereditary essential tremor
PharmGKBiPA28425

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiENOG410IP58 Eukaryota
ENOG4111GEG LUCA
GeneTreeiENSGT00940000157290
HOGENOMiHOG000038010
HOVERGENiHBG095056
InParanoidiP35637
KOiK13098
PhylomeDBiP35637
TreeFamiTF322599

Enzyme and pathway databases

ReactomeiR-HSA-72163 mRNA Splicing - Major Pathway
SIGNORiP35637

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
FUS human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
FUS

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
2521

Protein Ontology

More...
PROi
PR:P35637

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000089280 Expressed in 238 organ(s), highest expression level in right testis
CleanExiHS_FUS
ExpressionAtlasiP35637 baseline and differential
GenevisibleiP35637 HS

Family and domain databases

Gene3Di3.30.70.330, 1 hit
InterProiView protein in InterPro
IPR034459 FUS
IPR012677 Nucleotide-bd_a/b_plait_sf
IPR035979 RBD_domain_sf
IPR000504 RRM_dom
IPR034870 TET_fam
IPR001876 Znf_RanBP2
IPR036443 Znf_RanBP2_sf
PANTHERiPTHR23238 PTHR23238, 1 hit
PTHR23238:SF5 PTHR23238:SF5, 1 hit
PfamiView protein in Pfam
PF00076 RRM_1, 1 hit
PF00641 zf-RanBP, 1 hit
SMARTiView protein in SMART
SM00360 RRM, 1 hit
SM00547 ZnF_RBZ, 1 hit
SUPFAMiSSF54928 SSF54928, 1 hit
SSF90209 SSF90209, 1 hit
PROSITEiView protein in PROSITE
PS50102 RRM, 1 hit
PS01358 ZF_RANBP2_1, 1 hit
PS50199 ZF_RANBP2_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiFUS_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P35637
Secondary accession number(s): Q9H4A8
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 1, 1994
Last modified: December 5, 2018
This is version 210 of the entry and version 1 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  2. SIMILARITY comments
    Index of protein domains and families
  3. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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