在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. SPATA7 Antibody

SPATA7 Antibody

  • 中文名稱：
    SPATA7兔多克隆抗體
  • 貨號：
    CSB-PA195430
  • 規(guī)格：
    ￥1100
  • 圖片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA195430(SPATA7 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using CSB-PA195430(SPATA7 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

產(chǎn)品詳情

  • Uniprot No.：
  • 基因名：
    SPATA7
  • 別名：
    SPATA7 antibody; HSD3 antibody; Spermatogenesis-associated protein 7 antibody; HSD-3.1 antibody; Spermatogenesis-associated protein HSD3 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Fusion protein of Human SPATA7
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen affinity purification
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 產(chǎn)品提供形式：
    Liquid
  • 應用范圍：
    ELISA,IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:50-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

  • 功能：
    Involved in the maintenance of both rod and cone photoreceptor cells. It is required for recruitment and proper localization of RPGRIP1 to the photoreceptor connecting cilium (CC), as well as photoreceptor-specific localization of proximal CC proteins at the distal CC. Maintenance of protein localization at the photoreceptor-specific distal CC is essential for normal microtubule stability and to prevent photoreceptor degeneration.
  • 基因功能參考文獻：
    1. Compound heterozygous c.1100A > G, p.(Y367C) and c.1102_1103delCT, p.(L368Efs*4) variants in SPATA7 manifest as an unusual RP phenotype in this case, showing extensive choroidal sclerosis and retinal pigment epithelium (RPE) atrophy with evidence of progression over two years on multimodal imaging. PMID: 29411205
    2. We present the clinical and genetic findings of two siblings harboring the c.1112T>C/p.I371T homozygous mutation in the SPATA7 gene. PMID: 28481129
    3. The disease resulting from SPATA7 mutations in this patient initially presented as a cone-rod dystrophy (CRD), but changed over time into a phenotype more reminiscent of late-stage retinitis pigmentosa (RP). PMID: 26854980
    4. SPATA7 plays a role in RPGRIP1-mediated protein trafficking across the connecting cilium of photoreceptor cells. Apoptotic degeneration of these cells triggered by protein mislocalization is a mechanism of disease progression in LCA3/juvenile RP patients PMID: 25398945
    5. A novel homozygous large deletion in SPATA7 associated with juvenile retinitis pigmentosa has been found in a consanguineous Israeli Muslim Arab family. PMID: 25814828
    6. Digenic and triallelic mutations of CRB1 and SPATA7 were detected in a Chinese family with Leber congenital amaurosis. The results imply that CRB1 and SPATA7 may not interact with each other directly. PMID: 22219627
    7. In conclusion, our data established the first linkage association of a loss-of-function mutation in the SPATA7 gene with a typical retinitis pigmentosa (RP) phenotype and not with leber congenital amaurosis or early onset RP. PMID: 22136677
    8. Mutations in SPATA7 are a rare cause of childhood retinal dystrophy accounting for 1.7% of disease in this cohort. PMID: 21310915
    9. analysis of the SPATA7 mutations in Leber congenital amaurosis and the associated phenotype PMID: 20104588
    10. isolation and characterization of HSD-3.1 expressed in the testis PMID: 12736779
    11. Spata7 is expressed in the mature mouse retina. PMID: 19268277

    顯示更多

    收起更多

  • 相關(guān)疾病：
    Leber congenital amaurosis 3 (LCA3); Retinitis pigmentosa autosomal recessive (ARRP)
  • 亞細胞定位：
    Cytoplasm, cytoskeleton, cilium axoneme. Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton. Cell projection, cilium, photoreceptor outer segment.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 20423

    OMIM: 268000

    KEGG: hsa:55812

    STRING: 9606.ENSP00000377176

    UniGene: Hs.525518



主站蜘蛛池模板： 精品国产成人高清在线观看| 亚洲大成色www永久网站动图| 久久精品国产只有精品2020| 成人国产精品一区二区免费看| 中文精品久久久久国产网址| 国产精品无码mv在线观看| 水蜜桃精品一二三| 最美女人体内射精一区二区| 成人免费无码大片a毛片| 麻豆国产97在线 | 欧美| 华人在线亚洲欧美精品| 亚洲色无码中文字幕手机在线| 精品国产天线2019| 久久久精品人妻一区二区三区| 国产精品高清一区二区不卡片| 人妻系列无码专区免费视频| 国模丽丽啪啪一区二区| 麻批好紧日起要舒服死了| 日本中文字幕乱码aa高清电影| 欧美精品无码久久久久久| 无码一区二区| 中国熟妇牲交视频免费| 午夜免费啪视频在线观看区 | 无码少妇一区二区三区浪潮av| 亚洲欧美国产va在线播放 | 任你干视频精品播放| 国产无遮挡又爽又黄的视频| 久久不见久久见免费影院www | 亚洲国产制服丝袜高清在线| 国产免费无码一区二区三区| 韩国精品一区二区三区四区| 在线麻豆精东9制片厂av影现网| 日本无码人妻一区二区色欲| 45分钟免费真人视频| 性色a∨人人爽网站hdkp885| 久久国产亚洲精选av| 中文字幕丰满孑伦无码专区| 西西人体大胆瓣开下部毛茸茸| 日本三级欧美三级人妇视频| 国产成人午夜精华液| 亚洲天堂2017无码|