FITC标记的PDZ结构域PDZK7蛋白抗体
产品名称： FITC标记的PDZ结构域PDZK7蛋白抗体
英文名称： Anti-PDZD7/FITC
产品编号： HZ-9042R-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： IF=1:50-200
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Rabbit Anti-PDZD7/FITC Conjugated antibody
FITC标记的PDZ结构域PDZK7蛋白抗体
| 产品编号 | bs-9042R-FITC |
| 英文名称 | Anti-PDZD7/FITC |
| 中文名称 | FITC标记的PDZ结构域PDZK7蛋白抗体 |
| 别 名 | PDZ domain containing 7; PDZK7; RP11-108L7.9; EG435601; OTTMUSP00000044305; 9130207N01; OTTMUSP00000044304; PDZD7_HUMAN. |
| 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 免疫学 神经生物学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, 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 | 56kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PDZD7 |
| 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: PDZK7, also known as PDZD7, is a 517 amino acid protein that contains two PDZ (DHR) domains. Encoded by a gene that maps to human chromosome 10q24.31, PDZK7 is conserved in dog, mouse and rat, and exists as three alternatively spliced isoforms. PDZK7 is known to interact with Harmonin, MASS1, USH1G and Usherin. Localizing to nucleus, PDZK7 is expressed in retinal pigment epithelium and inner ear. Biallelic inactivation of PDZK7 can cause non-syndromic hearing impairment and chromosomal aberrations, which are linked to non-syndromic sensorineural deafness. PDZK7 mutations are also linked to Usher syndrome, which is characterized by retinitis pigmentosa and sensorineural deafness, and Alzheimer disease. The gene that encodes PDZK7 maps to human chromosome 10q24.31. Subunit: Interacts with USH1G. Interacts with GPR98. Interacts with USH2A. Subcellular Location: Cell projection; cilium. Nucleus. Tissue Specificity: Weakly expressed in the inner ear. Expressed in the retinal pigment epithelium. DISEASE: Note=A chromosomal aberration disrupting PDZD7 has been found in patients with non-syndromic sensorineural deafness. Translocation t(10;11),t(10;11). Defects in PDZD7 are a cause of Usher syndrome type 2C (USH2C) [MIM:605472]. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa with sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH2 is characterized by congenital mild hearing impairment with normal vestibular responses. Note=PDZD7 mutations have been found in combination with mutations in USH2A and GPR98 in patients affected by Usher syndrome, suggesting a role as contributor to digenic Usher syndrome or a modifier of retinal disease expression. Similarity: Contains 2 PDZ (DHR) domains. Database links: Entrez Gene: 79955 Human Omim: 612971 Human SwissProt: Q9H5P4 Human Unigene: 438245 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
PDZK7，也称为PDZD7，是包含两个PDZ（DHR）结构域的517氨基酸蛋白。由编码到人类染色体10q24.31的基因编码的PDZK7在狗、小鼠和大鼠中是保守的，并以三种交替剪接的亚型存在。PDZK7已知与Harmonin、Mas1、Us1G和UsHin相互作用。PDZK7定位于细胞核，表达于视网膜色素上皮和内耳。PDZK7双等位基因失活可引起非综合征性听力损害和染色体畸变，这与非综合征性感音神经性耳聋有关。PDZK7突变也与UHER综合征相关，其特征为视网膜色素变性和感音神经性耳聋和阿尔茨海默病。编码PDZK7的基因定位于人类染色体10q24.31。