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FUS Antibody

  • 货号：
    CSB-PA229936
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human breast cancer tissue using CSB-PA229936(FUS Antibody) at dilution 1/45, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    P35637
  • 基因名：
    FUS
  • 别名：
    75 kDa DNA pairing protein antibody; 75 kDa DNA-pairing protein antibody; ALS6 antibody; Amyotrophic lateral sclerosis 6 antibody; fus antibody; FUS CHOP antibody; Fus like protein antibody; FUS_HUMAN antibody; FUS1 antibody; Fused in sarcoma antibody; Fusion (involved in t(12,16) in malignant liposarcoma) antibody; Fusion derived from t(12,16) malignant liposarcoma antibody; Fusion gene in myxoid liposarcoma antibody; Heterogeneous nuclear ribonucleoprotein P2 antibody; hnRNP P2 antibody; hnRNPP2 antibody; Oncogene FUS antibody; Oncogene TLS antibody; POMp75 antibody; RNA binding protein FUS antibody; RNA-binding protein FUS antibody; TLS antibody; TLS CHOP antibody; Translocated in liposarcoma antibody; Translocated in liposarcoma protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human FUS
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:10000
    IHC 1:30-1:150
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    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. 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. Binds also its own pre-mRNA and autoregulates its expression; this autoregulation mechanism is mediated by non-sense-mediated decay. Plays a role in DNA repair mechanisms by promoting D-loop formation and homologous recombination during DNA double-strand break repair. In neuronal cells, plays crucial roles in dendritic spine formation and stability, RNA transport, mRNA stability and synaptic homeostasis.
  • 基因功能参考文献：
    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. s 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

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  • 相关疾病：
    Angiomatoid fibrous histiocytoma (AFH); Amyotrophic lateral sclerosis 6, with or without frontotemporal dementia (ALS6); Tremor, hereditary essential 4 (ETM4)
  • 亚细胞定位：
    Nucleus.
  • 蛋白家族：
    RRM TET family
  • 组织特异性：
    Ubiquitous.
  • 数据库链接：

    HGNC: 4010

    OMIM: 137070

    KEGG: hsa:2521

    STRING: 9606.ENSP00000254108

    UniGene: Hs.46894