Human RNA-binding protein FUS (FUS/TLS) ELISA kit

Instructions
Code CSB-E17376h
Size 96T,5×96T,10×96T
See More Details 24T ELISA kits trial application
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Product Details

Target Name fusion (involved in t(12;16) in malignant liposarcoma)
Alternative Names 75 kDa DNA pairing protein ELISA Kit; 75 kDa DNA-pairing protein ELISA Kit; ALS6 ELISA Kit; Amyotrophic lateral sclerosis 6 ELISA Kit; fus ELISA Kit; FUS CHOP ELISA Kit; Fus like protein ELISA Kit; FUS_HUMAN ELISA Kit; FUS1 ELISA Kit; Fused in sarcoma ELISA Kit; Fusion (involved in t(12,16) in malignant liposarcoma) ELISA Kit; Fusion derived from t(12,16) malignant liposarcoma ELISA Kit; Fusion gene in myxoid liposarcoma ELISA Kit; Heterogeneous nuclear ribonucleoprotein P2 ELISA Kit; hnRNP P2 ELISA Kit; hnRNPP2 ELISA Kit; Oncogene FUS ELISA Kit; Oncogene TLS ELISA Kit; POMp75 ELISA Kit; RNA binding protein FUS ELISA Kit; RNA-binding protein FUS ELISA Kit; TLS ELISA Kit; TLS CHOP ELISA Kit; Translocated in liposarcoma ELISA Kit; Translocated in liposarcoma protein ELISA Kit
Abbreviation FUS
Uniprot No. P35637
Species Homo sapiens (Human)
Sample Types serum, plasma, cell lysates
Detection Range 15.6 pg/mL-1000 pg/mL
Sensitivity 3.9 pg/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Signal Transduction
Precision
Linearity
Recovery
Typical Data
Troubleshooting
and FAQs
ELISA kit FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 3-5 working days

Target Data

Function 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.
Gene References into Functions
  1. loss of nuclear FUS caused DNA nick ligation defects in motor neurons. PMID: 30206235
  2. The herein presented data uncover a novel mechanism by which the fusion oncogene FUS-CHOP actively promotes invasion in myxoid and round cell liposarcoma through the activation of a SRC/FAK/RHO/ROCK signaling axis. PMID: 29190494
  3. When FUS was overexpressed and then de novo synthesis was blocked with ActD, the decay rate of LATS1/2 was slower in the FUS-overexpressed cells than in control cells. PMID: 30308519
  4. Motor neuron cultures exposed to mutant FUS (mutFUS)conditioned medium (ACM), but not wild-type FUS ACM, undergo significant cell loss, which is preceded by progressive degeneration of neurites. We found that Tumor TNFalpha is secreted into ACM of mutFUS-expressing astrocytes. Accordingly, mutFUS astrocyte-mediated motor neuron toxicity is blocked by targeting soluble TNFalpha with neutralizing antibodies. PMID: 29380416
  5. the abnormal stable complex of FUS-R521C/PRMT1/Nd1-L mRNA could contribute to neurodegeneration upon oxidative stress. PMID: 28094300
  6. more selective group of neurons appears to be affected in frontotemporal lobar degeneration (FTLD)-TDP and FTLD-FUS than in FTLD-tau PMID: 28984110
  7. Taken together, FUS RNA-recognition motif appears to play a crucial role in exaggerating the physiological/reversible self-assembly into pathological/irreversible fibrillization, thus contributing to manifestation of FUS cytotoxicity. PMID: 28432364
  8. Fus is a binding partner of FMRP. PMID: 28424484
  9. Study demonstrates that FUS mutants, but not WT forms, impair fast axonal transport (FAT) in brain tissue of patients with ALS, through a mechanism dependent on activation of p38 MAPK. PMID: 28273913
  10. In human stem cell-derived motor neurons, the RNA profile associated with concomitant loss of both TAF15 and FUS resembles that observed in the presence of the amyotrophic lateral sclerosis (ALS)-associated mutation FUS R521G, but contrasts with late-stage sporadic ALS patients. PMID: 27378374
  11. FUS P525L mutation alters transcriptome and microRNA pathways in motor neurons with implications for ALS pathogenesis. PMID: 28988989
  12. This study showed that in fibroblasts of FUS P525L mutation carriers, FUS mislocalized to the cytoplasm where it redistributed into stress granules with likely a dose effect. PMID: 29035885
  13. Results find that mutant but not wild-type FUS decreased dendritic growth, mRNA levels, and protein synthesis in dendrites. These data suggest that cytoplasmic FUS aggregates trap mRNA and its transporters, impairing dendritic mRNA trafficking and translation, in turn leading to the disruption of dendritic homeostasis and the development of frontotemporal dementia phenotypes. PMID: 28928015
  14. activation of the IGF-IR/PI3K/Akt signaling system is a common pattern in MLS which appears to be transcriptionally controlled, at least in part by induction of IGF2 gene transcription in a FUS-DDIT3-dependent manner. PMID: 28637688
  15. SOD1 mutations were present in 20% of familial amyotrophic lateral sclerosis (ALS) patients and 1.9% of sporadic ALS patients, while FUS mutations were responsible for 13.3% of familial ALS cases, and TARDBP mutations were rare in either familial or sporadic ALS cases. PMID: 27604643
  16. Depletion of SAFB1 reduced FUS's localization to chromatin-bound fraction and splicing activity, suggesting SAFB1 could tether FUS to chromatin compartment thorough N-terminal DNA-binding motif. Moreover, FUS interacts with another nuclear matrix-associated protein, Matrin3. PMID: 27731383
  17. A molecular docking and dynamics study concluded that R521C and R521H mutations in FUS result in weak binding with Karyopherin-beta2 leading to amyotrophic lateral sclerosis. PMID: 27381509
  18. both FUS and TDP43 colocalize with active RNA polymerase II at sites of DNA damage along with the DNA damage repair protein, BRCA1, and FUS and TDP43 participate in the prevention or repair of R loop-associated DNA damage, a manifestation of aberrant transcription and/or RNA processing PMID: 27849576
  19. FUS mutations were significantly more common among mainland Chinese patients than those among Caucasian populations (p=6.8x10-3). The high frequency of FUS mutations in FALS and SALS in mainland China is another genetic feature distinct from Caucasians. PMID: 26519472
  20. The impairment of PARP-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation in amyotrophic lateral sclerosis. PMID: 29362359
  21. ALS-associated mutations enhance FUS protein propagation in Drosophila neurons. PMID: 28429234
  22. juvenile ALS linked to FUS mutations represent a specific entity different from both classical juvenile ALS and adult ALS linked to FUS gene PMID: 28054830
  23. Motor neurons expressing FUS with the P525L or the R521H mutation showed cytoplasmic mislocalization of FUS, hypoexcitability, and axonal transport defects. PMID: 29021520
  24. Results suggest that RBM45 serves as a negative regulator to prevent FUS-mediated excessive recruitment of HDAC1 to the sites of DNA damage. PMID: 29140459
  25. The review describes the main physiological functions of FUS and considers evidence for each of the theories of amyotrophic lateral sclerosis pathogenesis. PMID: 28707655
  26. Authors used solid-state nuclear magnetic resonance methods to characterize the molecular structure of self-assembling fibrils formed by the LC domain of the fused in sarcoma (FUS) RNA-binding protein. From the 214-residue LC domain of FUS (FUS-LC), a segment of only 57 residues forms the fibril core, while other segments remain dynamically disordered. PMID: 28942918
  27. Nuclear magnetic resonance spectroscopy demonstrates the intrinsically disordered structure of FUS's nearly uncharged, aggregation-prone, yeast prion-like, low sequence-complexity domain is preserved after phosphorylation. PMID: 28790177
  28. Long noncoding RNA SchLAH functions through interaction with fused in sarcoma protein (FUS). PMID: 28196303
  29. Focus on the recent advances on approaches to uncover the mechanisms of wild type and mutant FUS proteins during development and in neurodegeneration (review). PMID: 27033831
  30. FUS-induced reductions to ER-mitochondria associations and are linked to activation of glycogen synthase kinase-3beta (GSK-3beta), a kinase already strongly associated with ALS/FTD. PMID: 27418313
  31. Motor-neuron disease (MND)-linked RNA-binding proteins (RBPs), TDP-43, FUS, and hnRNPA2B1, bind to and induce structural alteration of UGGAAexp. These RBPs suppress UGGAAexp-mediated toxicity in Drosophila by functioning as RNA chaperones for proper UGGAAexp folding and regulation of pentapeptide repeat translation. PMID: 28343865
  32. we analyzed fast axonal transport in larval motor neurons of Drosophila models of TARDBP (TDP-43), FUS and C9orf72. We also analyzed the effect of loss-of-function mutants of the Drosophila orthologs of TDP-43 and FUS, TBPH and caz, respectively. The motor activities of larvae and adults in these models were assessed to correlate potential defects in axonal transport with locomotor deficits PMID: 27056981
  33. These findings suggest a possible pathomechanism for amyotrophic lateral sclerosis in which mutated FUS inhibits correct splicing of minor introns in mRNAs encoding proteins required for motor neuron survival. PMID: 27252488
  34. This study revealed a characteristic phenotype in FUS/TLS-linked FALS patients in Japan. PMID: 26823199
  35. The aim of this review will be to provide a general overview of TDP-43 and FUS/TLS proteins and to highlight their physiological functions--{REVIEW} PMID: 27015757
  36. Mouse model that overexpresses FUS without a nuclear localization signal (DeltaNLS-FUS) shows progressive motor deficits/ALS phenotype PMID: 27368346
  37. Possible role of deregulated DNA binding function of FUS in ALS. PMID: 27693252
  38. Our in vivo studies of the hFUS-Q290X mutation in Drosophila link motor dysfunction to impairment in the GABAergic pathway. Our findings would facilitate further efforts in unravelling the pathophysiology of Essential tremor PMID: 27395408
  39. FUS is glycosylated with a high stoichiometry not only in the neural cells but also in the non-neural cell lines. PMID: 27903134
  40. The results of this study suggested that FUS mutations are the most frequent genetic cause in early-onset sporadic ALS patients of Chinese origin. PMID: 26972116
  41. Data show that induced pluripotent stem cells (iPSC)-derived motor neurons mimicked several neurodegenerative phenotypes including mis-localization of fused-in sarcoma (FUS) gene product into cytosolic. PMID: 26997647
  42. miR-141 and the FUS gene, which are inversely correlated, play significant functional roles in regulating human neuroblastoma. PMID: 26936280
  43. pathological TDP-43 and FUS may exert motor neuron pathology in amyotrophic lateral sclerosis through the initiation of propagated misfolding of SOD1 PMID: 26926802
  44. iNeurons may provide a more reliable model for investigating FUS mutations with disrupted NLS for understanding FUS-associated proteinopathies in amyotrophic lateral sclerosis PMID: 26795035
  45. A subset of juvenile-onset familial/sporadic ALS cases with FUS gene mutations reportedly demonstrates mental retardation or learning difficulty. PMID: 26984092
  46. Study implicates phosphorylation as an additional mechanism by which nuclear transport of FUS might be regulated and potentially perturbed in ALS and FTLD. PMID: 26403203
  47. Study identifies a common mechanism of transport into neurites of proteins linked to the pathology of Alzheimer's disease (i.e. sAPP) and ALS (i.e. FUS, TDP-43 and SOD1) PMID: 26605911
  48. RNA binding proteins TDP-43 and FUS do not consistently fit the currently characterised inclusion models suggesting that cells have a larger repertoire for generating inclusions than currently thought. PMID: 26293199
  49. Wild-type and mutant hFUS proteins induced neuronal degeneration with partial selectivity for motor neurons. Motor neuron loss was accompanied by abnormal neurite morphology and length. PMID: 26174443
  50. FUS mutation seems indicated in sporadic early-onset ALS patients especially if showing predominant bulbar symptoms and an aggressive disease course. PMID: 26362943
  51. Results demonstrate that ALS-mutant forms of TDP-43, FUS, and SOD1 all perturb protein transport in the early secretory pathway between ER and Golgi compartments, and imply that restoring Rab1-mediated ER-Golgi transport is a novel ALS therapeutic target PMID: 26298469
  52. FUS-DDIT3 is uniquely regulated at the transcriptional as well as the post-translational level and that its expression level is important for myxoid liposarcoma tumour development. PMID: 26865464
  53. FUS-dependent motor degeneration is not due to loss of FUS function, but to the gain of toxic properties conferred by ALS mutations. PMID: 26842965
  54. FUS1 has roles in cancer, inflammation, and autoimmunity [review] PMID: 26246474
  55. Genetic polymorphisms in the TLS genes might serve as potential predictive biomarkers of prognosis of advanced NSCLC patients treated with platinum-based chemotherapy. PMID: 26611653
  56. Biochemical and genetic data demonstrate that FUS interacts with a mitochondrial chaperonin, HSP60, and that FUS translocation to mitochondria is, at least in part, mediated by HSP60 PMID: 26335776
  57. This study confirms cases of ALS-FUS to be mainly a lower motor neuron disease and to have pathology that does not appear to neatly correlate with clinical features or genetics. PMID: 26452761
  58. aberrant localization and recruitment of FUS into stress granules is a prerogative of the FUS mutant proteins and occurs only upon induction of stress in both undifferentiated iPSCs and spinal cord neural cells PMID: 26035390
  59. All the results demonstrated that FUS1 acts as a tumor-suppressor gene by upregulating miR-197 in human glioblastoma and implied that restoration of FUS1 and miR-197 could be new therapeutic strategies for glioblastoma. PMID: 26081814
  60. due to mutation, FUS protein became more rigid in nature and might alter the structural and functional behavior of protein and play a major role in inducing Amyotrophic lateral sclerosis 6 PMID: 24738488
  61. Oxr1 serves as a potential therapeutic target for ALS and other neurodegenerative disorders characterized by TDP-43 or FUS pathology. PMID: 25792726
  62. these studies establish potentially converging disease mechanisms in amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy, with ALS-causative mutants acquiring properties representing both gain and loss of function. PMID: 25625564
  63. TDP-43 and FUS were found to regulate splicing and expression of genes related to neuronal (SEPT6, SULT4A1, TNIK) and RNA metabolism (DICER, ELAVL3/HuC, POLDIP3). PMID: 26514432
  64. FUS low-complexicity phase separates to recruit RNA polymerase II C-terminal domain in vitro. PMID: 26455390
  65. Results that the molecular mechanisms controlling the nuclear import of FUS/Caz and FUS autoregulation are conserved between humans and Drosophila PMID: 25447237
  66. THe findings of this study provided no evidence for a role of rare genetic variants in the pathogenesis of ET, apart from the initially published FUS mutation segregating in a large ET family. PMID: 25631824
  67. The study concludes that FUS has quite general nucleic acid-binding activity, with the various proposed RNA motifs being neither necessary for FUS binding nor sufficient to explain its diverse binding partners. PMID: 26150427
  68. Using an in vitro "aging" experiment, study demonstrates that liquid droplets of FUS protein convert with time from a liquid to an aggregated state, and this conversion is accelerated by patient-derived mutations; authors conclude that the physiological role of FUS requires forming dynamic liquid-like compartments. PMID: 26317470
  69. de novo FUS mutations are responsible for a certain proportion of sALS. PMID: 25457557
  70. FUS mutation identified in amyotrophic lateral sclerosis patients maps to the sequence recognized by miR-141 and miR-200a. PMID: 25004804
  71. A small subset of Myoepithelial tumors harbor FUS rearrangements, two thirds of them being associated with KLF17 fusion. PMID: 25706482
  72. ALS mutations in FUS can strongly impact target gene expression, reflecting a dominant effect of FUS-containing aggregates. PMID: 26251528
  73. data lead to a model that FUS functions in coupling transcription to splicing via mediating an interaction between RNAP II and U1 snRNP PMID: 26124092
  74. Our results suggested that miR-378 enhanced cell migration and metastasis by down-regulating Fus expression. PMID: 25562172
  75. Observations suggest that the gain-of-function of FUS in the nucleus contributes to the pathogenesis of FUS-linked neurodegenerative diseases. PMID: 25497700
  76. It involves in metabolic and protein degradation pathways. PMID: 25192599
  77. Loss of FUS expression severely impairs cellular proliferation and leads to an increase in phosphorylated histone H3. PMID: 25501833
  78. This study demonstrated that interaction of multiple domains including intronic domains are involved in expression of FUS. PMID: 25451114
  79. A novel association of common variable immunodeficiency have been found with rare variants at the FUS/ITGAM (CD11b) locus on 16p11.2. PMID: 25678086
  80. This study provides further evidence of a broad correlation between clinical severity of FUS-related ALS and mislocalization of the protein to the cytoplasm. PMID: 24899262
  81. Study provides evidence for loss of PRMT1 function as a consequence of cytoplasmic accumulation of FUS in the pathogenesis of amyotrophic lateral sclerosis, including changes in the histone code regulating gene transcription. PMID: 25274782
  82. Suggest that cationic liposome-mediated FUS1-hIL-12 coexpression may be a new promising strategy for lung cancer. PMID: 24410728
  83. components of the U1 snRNP core particle (Sm proteins and U1 snRNA), but not the mature U1 snRNP-specific proteins (U1-70K, U1A and U1C), co-mislocalize with FUS to the cytoplasm in ALS fibroblasts with mutations in the FUS nuclear localization signal PMID: 25735748
  84. subcellular localization of human FUS and the generation of stress granule PMID: 24912067
  85. our study provided evidence that a multistep process of FUS aggregation in the cell cytoplasm includes RNA-dependent and RNA-independent mechanisms. PMID: 24842888
  86. Altered expression of FUS is associated with inappropriate phosphorylation of RNA Pol II. PMID: 25009283
  87. FUS mutations linked with neurogeneration on Guam. PMID: 25558820
  88. Self-assembled FUS binds active chromatin and regulates gene transcription. PMID: 25453086
  89. Aggregation of FUS mutant sequesters RNA binding proteins and impairs RNA granules formation. PMID: 25173930
  90. Taken together, FUS autoregulation by alternative splicing provides insight into a molecular mechanism by which FUS-regulated pre-mRNA processing can impact a significant number of targets important to neurodegeneration. PMID: 24204307
  91. Data demonstrate that FUS/Caz regulates neuromuscular junction development and plays an evolutionarily conserved role in modulating the strength of synaptic transmission in motor neurons. PMID: 24569165
  92. A primate specific and polymorphic retrotransposon can act as a classical transcriptional regulatory domain upstream of the FUS gene. PMID: 24608899
  93. HuR regulates TDP-43 and FUS/TLS expression and loss of HuR-mediated RNA processing in astrocytes can alter the molecular and cellular landscape to produce a toxic phenotype PMID: 25239623
  94. our data suggest that TAF15 and TLS/FUS operate within similar but not identical hnRNP M-TET protein complexes to influence the transcriptional or post-transcriptional output of a particular cell type. PMID: 24474660
  95. This study has identified a novel FUS p.R377W substitution in essential tremor patients. PMID: 23834483
  96. FUS plays a critical role in MnSOD gene transcription. PMID: 23834335
  97. Review summarizes current understanding of the normal function of FUS, and describes its role in the pathology of ALS, FTLD, essential tremor and other neurodegenerative diseases. [review] PMID: 24840975
  98. It is one of the most common ALS related genes. PMID: 24325798
  99. The FUS gene is not a genetic risk factor for Parkinson's disease in the population of Chinese Han ethnicity. PMID: 24080306
  100. REVIEW: data on FUS aggregation signatures in available model organisms on the basis of overexpression of FUS variants PMID: 24256263

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Involvement in disease Angiomatoid fibrous histiocytoma (AFH); Amyotrophic lateral sclerosis 6, with or without frontotemporal dementia (ALS6); Tremor, hereditary essential 4 (ETM4)
Subcellular Location Nucleus
Protein Families RRM TET family
Tissue Specificity Ubiquitous.
Database Links

HGNC: 4010

OMIM: 137070

KEGG: hsa:2521

STRING: 9606.ENSP00000254108

UniGene: Hs.46894

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