| 產(chǎn)品編號(hào) | bs-2980R-BF594 |
| 英文名稱1 | Rabbit Anti-TLS/FUS/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的粘液樣脂肪肉瘤蛋白FUS1抗體 |
| 別 名 | 75 kDa DNA pairing protein; 75 kDa DNA-pairing protein; ALS6; Amyotrophic lateral sclerosis 6; FUS; FUS CHOP; Fus like protein; FUS-CHOP; FUS_HUMAN; FUS1; Fused in sarcoma; Fusion (involved in t(12;16) in malignant liposarcoma); Fusion derived from t(12;16) malignant liposarcoma; Fusion gene in myxoid liposarcoma; Heterogeneous nuclear ribonucleoprotein P2; hnRNP P2; hnRNPP2; Oncogene FUS; Oncogene TLS; POMp75; RNA binding protein FUS; RNA-binding protein FUS; TLS; TLS CHOP; TLS-CHOP; Translocated in liposarcoma; Translocated in liposarcoma protein. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 免疫學(xué) 染色質(zhì)和核信號(hào) 信號(hào)轉(zhuǎn)導(dǎo) 轉(zhuǎn)錄調(diào)節(jié)因子 表觀遺傳學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 53kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human TLS |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: This gene encodes a multifunctional protein component of the heterogeneous nuclear ribonucleoprotein (hnRNP) complex. The hnRNP complex is involved in pre-mRNA splicing and the export of fully processed mRNA to the cytoplasm. This protein belongs to the FET family of RNA-binding proteins which have been implicated in cellular processes that include regulation of gene expression, maintenance of genomic integrity and mRNA/microRNA processing. Alternative splicing results in multiple transcript variants. Defects in this gene result in amyotrophic lateral sclerosis type 6. [provided by RefSeq]. 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. Subunit: Component of nuclear riboprotein complexes. Interacts with ILF3, TDRD3 and SF1. Interacts through its C-terminus with SFRS13A. Interacts with OTUB1 and SARNP. Subcellular Location: Nucleus. Tissue Specificity: Ubiquitous. Post-translational modifications: Arg-216 and Arg-218 are dimethylated, probably to asymmetric dimethylarginine. DISEASE: Note=A chromosomal aberration involving FUS is found in a patient with malignant myxoid liposarcoma. Translocation t(12;16)(q13;p11) with DDIT3. Note=A chromosomal aberration involving FUS is a cause of acute myeloid leukemia (AML). Translocation t(16;21)(p11;q22) with ERG. Angiomatoid fibrous histiocytoma (AFH) [MIM:612160]: A 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. Note=The disease may be caused by mutations affecting the gene represented in this entry. 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. Amyotrophic lateral sclerosis 6 (ALS6) [MIM:608030]: A 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Tremor, hereditary essential 4 (ETM4) [MIM:614782]: A 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. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the RRM TET family. Contains 1 RanBP2-type zinc finger. Contains 1 RRM (RNA recognition motif) domain. Database links: Entrez Gene: 2521 Human Entrez Gene: 233908 Mouse Omim: 137070 Human SwissProt: P35637 Human SwissProt: P56959 Mouse Unigene: 46894 Human Unigene: 277680 Mouse Unigene: 100218 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
