| 產(chǎn)品編號(hào) | bs-2756R-BF594 |
| 英文名稱 | Rabbit Anti-CYLD/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的微管結(jié)合蛋白CYLD抗體 |
| 別 名 | CDMT; cylindromatosis (turban tumor syndrome); cylindromatosis 1; Deubiquitinating enzyme CYLD; EAC; HSPC057; KIAA0849; turban tumor syndrome; Ubiquitin thiolesterase CYLD; Ubiquitin-specific processing protease CYLD; CYLD_HUMAN; Ubiquitin carboxyl-terminal hydrolase CYLD; CYLD; BRSS; CDMT; CYLD1; CYLDI; EAC; MFT; MFT1; SBS; TEM; USPL2. |
| 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 細(xì)胞生物 細(xì)胞類型標(biāo)志物 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, Rat, (predicted: Human, Chicken, Cow, Horse, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 105kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human cylindromatosis 1 |
| 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: Defects in CYLD are the cause of familial cylindromatosis (CYLD) also known as turban tumor syndrome or dermal eccrine cylindromatosis. CYLD is an autosomal dominant and highly tumor type-specific disorder. The tumors (known as cylindromas because of their characteristic microscopic architecture) are believed to arise from or recapitulate the appearance of the eccrine or apocrine cells of the skin that secrete sweat and scent respectively. Cylindromas arise predominantly in hairy parts of the body with approximately 90% on the head and neck. The development of a confluent mass which may ulcerate or become infected has led to the designation "turban tumor syndrome". The skin tumors show differentiation in the direction of hair structures, hence the synonym trichoepithelioma. CYLD has deubiquitinating activity. Function: Protease that specifically cleaves 'Lys-63'-linked polyubiquitin chains. Has endodeubiquitinase activity. Plays an important role in the regulation of pathways leading to NF-kappa-B activation. Contributes to the regulation of cell survival, proliferation and differentiation via its effects on NF-kappa-B activation. Negative regulator of Wnt signaling. Inhibits HDAC6 and thereby promotes acetylation of alpha-tubulin and stabilization of microtubules. Plays a role in the regulation of microtubule dynamics, and thereby contributes to the regulation of cell proliferation, cell polarization, cell migration, and angiogenesis. Required for normal cell cycle progress and normal cytokinesis. Inhibits nuclear translocation of NF-kappa-B. Plays a role in the regulation of inflammation and the innate immune response, via its effects on NF-kappa-B activation. Dispensable for the maturation of intrathymic natural killer cells, but required for the continued survival of immature natural killer cells. Negatively regulates TNFRSF11A signaling and osteoclastogenesis Subunit: Interacts (via CAP-Gly domain) with IKBKG/NEMO (via proline-rich C-terminal region). Interacts with TRAF2 and TRIP. Interacts with PLK1, DVL1, DVL3, MAVS, TBK1, IKKE and DDX58. Interacts (via CAP-Gly domain) with microtubules. Interacts with HDAC6 and BCL3. Interacts with SQSTM1 and MAP3K7. Identified in a complex with TRAF6 and SQSTM1. Subcellular Location: Cytoplasm. Cytoplasm, perinuclear region. Cytoplasm, cytoskeleton. Cell membrane; Peripheral membrane protein; Cytoplasmic side. Note=Detected at the microtubule cytoskeleton during interphase. Detected at the midbody during telophase. Tissue Specificity: Detected in fetal brain, testis, and skeletal muscle, and at a lower level in adult brain, leukocytes, liver, heart, kidney, spleen, ovary and lung. Isoform 2 is found in all tissues except kidney. Post-translational modifications: Phosphorylated on several serine residues by IKKA and/or IKKB in response to immune stimuli. Phosphorylation requires IKBKG. Phosphorylation abolishes TRAF2 deubiquitination, interferes with the activation of Jun kinases, and strongly reduces CD40-dependent gene activation by NF-kappa-B. DISEASE: Familial cylindromatosis (FCYL) [MIM:132700]: Autosomal dominant and highly tumor type-specific disorder. The tumors (known as cylindromas because of their characteristic microscopic architecture) are believed to arise from or recapitulate the appearance of the eccrine or apocrine cells of the skin that secrete sweat and scent respectively. Cylindromas arise predominantly in hairy parts of the body with approximately 90% on the head and neck. The development of a confluent mass which may ulcerate or become infected has led to the designation 'turban tumor syndrome'. The skin tumors show differentiation in the direction of hair structures, hence the synonym trichoepithelioma. Note=The disease is caused by mutations affecting the gene represented in this entry. Multiple familial trichoepithelioma 1 (MFT1) [MIM:601606]: Autosomal dominant dermatosis characterized by the presence of many skin tumors predominantly on the face. Since histologic examination shows dermal aggregates of basaloid cells with connection to or differentiation toward hair follicles, this disorder has been thought to represent a benign hamartoma of the pilosebaceous apparatus. Trichoepitheliomas can degenerate into basal cell carcinoma. Note=The disease is caused by mutations affecting the gene represented in this entry. Brooke-Spiegler syndrome (BRSS) [MIM:605041]: An autosomal dominant disorder characterized by the appearance of multiple skin appendage tumors such as cylindroma, trichoepithelioma, and spiradenoma. These tumors are typically located in the head and neck region, appear in early adulthood, and gradually increase in size and number throughout life. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the peptidase C67 family. Contains 3 CAP-Gly domains. Database links: Entrez Gene: 1540 Human Entrez Gene: 74256 Mouse Omim: 605018 Human SwissProt: Q9NQC7 Human SwissProt: Q80TQ2 Mouse Unigene: 578973 Human Unigene: 482446 Mouse Unigene: 128760 Rat Unigene: 168938 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. CYLD(cylindromatosis)是近年發(fā)現(xiàn)的一種腫瘤抑制基因,CYLD丟失或突變可致腫瘤形成,多個(gè)研究顯示,其表達(dá)蛋白CYLD可去泛素化TRAFs、NEMO、Bcl-3及p53等信號(hào)分子,調(diào)控細(xì)胞NF-κB和JNK等信號(hào)途徑,CYLD在人體內(nèi)廣泛分布，在細(xì)胞周期的調(diào)控、介導(dǎo)細(xì)胞凋亡、抑制腫瘤發(fā)生等分子事件中有著重要的調(diào)控作用。 CYLD基因缺陷或缺失主要導(dǎo)致頭部或面部的皮膚腫瘤,如多發(fā)性家族性毛發(fā)上皮瘤(MFT),家族性圓柱瘤(FC)和Brooke-Spiegler綜合癥(BSS),此外,CYLD在宮頸癌、腎癌、結(jié)腸癌、肝癌形成的信號(hào)途徑中也均表現(xiàn)為下游調(diào)控，是目前用于腫瘤研究的熱門抗體。 |
