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FITC标记的白血病相关蛋白AHI1抗体
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更新日期：2026/8/7 18:14:45
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相关标签：FITC标记的白血病相关蛋白AHI1抗体
详细内容
| 英文名称 | Anti-AHI1/FITC |
| 中文名称 | FITC标记的白血病相关蛋白AHI1抗体 |
| 别 名 | Abelson helper integration site 1 protein homolog; Abelson helper integration site 1; Abelson helper integration site; AHI 1; AHI-1; Ahi1; AHI1_HUMAN; Contatins SH3 ａnd WD40 domains; JBTS3; Jouberin; ORF1. |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 细胞周期蛋白 细胞分化 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 137kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human AHI1 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 |
| 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: Highly expressed in the most primitive normal hematopoietic cells. Expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract ａnd superior cerebellar peduncles. Expressed in kidney (renal collecting duct cells) (at protein level). Involvement in disease:Defects in AHI1 are the cause of Joubert syndrome type 3 (JBTS3) . JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities ａnd psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened ａnd reoriented superior cerebellar peduncles, ａnd an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy ａnd renal disease. JBTS3 shows minimal extra central nervous system involvement ａnd appears not to be associated with renal dysfunction. Function: Component of the tectonic-like complex, a complexlocalized at the transition zone of primary cilia ａnd acting as abarrier that prevents diffusion of transmembrane proteins betweenthe cilia ａnd plasma membranes (By similarity). Subunit: Part of the tectonic-like complex (also named B9complex). Interacts with MKS1 (By similarity). Interacts withNPHP1. Subcellular Location: Cytoplasm, cytoskeleton, cilium basal body(By similarity). Cell junction, adherens junction. Tissue Specificity: Highly expressed in the most primitive normalhematopoietic cells. Expressed in brain, particularly in neuronsthat give rise to the crossing axons of the corticospinal tract andsuperior cerebellar peduncles. Expressed in kidney (renalcollecting duct cells) (at protein level). |
FITC标记的白血病相关蛋白AHI1抗体
