FITC标记的跨膜蛋白48
产品名称: FITC标记的跨膜蛋白48
英文名称: Anti-NDC1/FITC
产品编号: HZ-19057R-FITC
产品价格: null
产品产地: 中国/上海
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: ICC=1:50-200 IF=1:50-200
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Rabbit Anti-NDC1/FITC Conjugated antibody
FITC标记的跨膜蛋白48
英文名称 | Anti-NDC1/FITC |
中文名称 | FITC标记的跨膜蛋白48 |
别 名 | nucleoporin NDC1; TMEM48; transmembrane protein 48; NET3. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul |
研究领域 | 细胞生物 染色质和核信号 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Cat, |
产品应用 | 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 | 75kDa |
性 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human NDC1 |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存条件 | 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. |
产品介绍 | background: NDC1 is a 674 amino acid multi-pass membrane protein, central core structure of the nuclear pore complex (NPC) and member of the NDC1 family that is crucial for selective nuclear protein import. Existing as four alternatively spliced isoforms that are encoded by a gene located on human chromosome 1, NDC1 interacts with Nup35 and anchors Aladin to the nuclear envelope of the NPC, a region of macromolecular transport between the nucleus and cytoplasm. In the absence of NDC1, Aladin becomes mislocalized and may lead to the development of an autosomal recessive disorder termed achalasia-addisonianism-alacrima (triple A) syndrome. Triple A syndrome is characterized by achalasia, alacrima and adrenocortico-tropin-resistant adrenal insufficiency. Robust expression in neural systems associated with cognitive, motor and sensory functions is consistent with the myriad of symptoms experienced by patients with triple A syndrome. Function: Component of the nuclear pore complex (NPC), which plays a key role in de novo assembly and insertion of NPC in the nuclear envelope. Required for NPC and nuclear envelope assembly, possibly by forming a link between the nuclear envelope membrane and soluble nucleoporins, thereby anchoring the NPC in the membrane. Subunit: Interacts with the NUP35/NUP53 By similarity. Interacts with AAAS, anchoring it to the nuclear envelope. Subcellular Location: Nucleus; nuclear pore complex; multipass membrane protein Similarity: Belongs to the NDC1 family. Database links: Entrez Gene: 55706 Human Entrez Gene: 72787 Mouse Entrez Gene: 362557 Rat Entrez Gene: 335236 Zebrafish Omim: 610115 Human SwissProt: Q9BTX1 Human SwissProt: Q8VCB1 Mouse SwissProt: Q6AXN4 Rat SwissProt: Q7SZC5 Zebrafish Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
NDC1是一种674氨基酸的多程膜蛋白,是核孔复合物(NPC)的中心核心结构,是NDC1家族成员,对选择性核蛋白导入至关重要。NDC1由位于人类1号染色体上的基因编码的四种可选择的剪接异构体存在,它与Nup35相互作用,将Aladin锚定到NPC的核膜上,NPC是细胞核和细胞质之间的大分子转运区域。在缺乏NDC1的情况下,Aladin变得错位,并可能导致称为贲门失弛缓症-阿迪逊-阿拉克里米亚(三A)综合征的常染色体隐性障碍的发展。三A综合征的特点是贲门失弛缓症,肾上腺皮质激素和肾上腺皮质激素抵抗的肾上腺皮质功能不全。神经系统中与认知、运动和感觉功能相关的强健表达与三A综合征患者经历的多种症状相一致。