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

WDR35 Antibody, Biotin conjugated

  • 中文名稱:
    WDR35兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA879071LD01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) WDR35 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    WDR35
  • 別名:
    Intraflagellar transport protein 121 homolog antibody; KIAA1336 antibody; MGC33196 antibody; Naofen antibody; WD repeat domain 35 antibody; WD repeat-containing protein 35 antibody; WDR35 antibody; WDR35_HUMAN antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human WD repeat-containing protein 35 protein (954-1181AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Biotin
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA
  • 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.

產品評價

靶點詳情

  • 功能:
    As a component of the IFT complex A (IFT-A), a complex required for retrograde ciliary transport and entry into cilia of G protein-coupled receptors (GPCRs), it is involved in ciliogenesis and ciliary protein trafficking. May promote CASP3 activation and TNF-stimulated apoptosis.
  • 基因功能參考文獻:
    1. Homozygous missense mutation in WDR35 gene is associated with multiple congenital anomalies, including brain malformations and skeletal dysplasia suggestive of cranioectodermal dysplasia ciliopathy. PMID: 29174089
    2. The observations of the Sensenbrenner syndrome patient in this study provide additional clinical data and expand the molecular spectrum of Sensenbrenner syndrome. Moreover, the two variants identified in the proband provide further evidence for the WDR35 mutations as the most common cause of this rare syndrome. PMID: 29134781
    3. A differential diagnosis of Sensenbrenner Syndrome was made after a novel homozygous missense mutation in WDR35 was identified in a patient with initial diagnosis of Jeune syndrome. PMID: 28870638
    4. Wdr35 regulates cilium assembly by selectively regulating transport of distinct cargoes. PMID: 27806291
    5. Psychomotor development was apparently normal. Molecular analysis in one of the affected individuals identified compound heterozygosity for a nonsense (c.1922T>G, p.(Leu641*)) and missense (c.2522A>T, p.(Asp841Val)) variants in WDR35. We PMID: 28332779
    6. Splicing variants in WDR35, and possibly in other IFT-A components, underlie a number of Ellis-van Creveld syndrome cases by disrupting targeting of both the EvC complex and Smoothened to cilia. PMID: 25908617
    7. report on the detection of novel WDR35 mutations in two unrelated cranioectodermal dysplasia patients PMID: 22486404
    8. A pathogenic WDR35 mutation was identified in a family with a complex clinical presentation that includes significant overlap of the phenotypes described in Sensenbrenner syndrome and the unclassified short-rib polydactyly syndromes. PMID: 22987818
    9. Through structural modeling, we show that WDR35 has strong homology to the COPI coatamers involved in vesicular trafficking and that short-rib polydactyly mutations affect key structural elements in WDR35. PMID: 21473986
    10. WDR35 is homologous to TULP4 (from the Tubby superfamily) and has previously been characterized as an intraflagellar transport component, confirming that Sensenbrenner syndrome is a ciliary disorder. PMID: 20817137
    11. These results indicated that naofen may function as a novel modulator activating caspase-3, and promoting TNF-alpha-stimulated apoptosis. PMID: 20193664

    顯示更多

    收起更多

  • 相關疾病:
    Cranioectodermal dysplasia 2 (CED2); Short-rib thoracic dysplasia 7 with or without polydactyly (SRTD7)
  • 亞細胞定位:
    Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, cilium axoneme. Cytoplasm, cytoskeleton, cilium basal body.
  • 數據庫鏈接:

    HGNC: 29250

    OMIM: 613602

    KEGG: hsa:57539

    STRING: 9606.ENSP00000314444

    UniGene: Hs.205427



主站蜘蛛池模板: 中文字幕人妻高清乱码| 欧美牲交a欧美牲交aⅴ| 欧亚乱熟女一区二区在线| 丰满爆乳无码一区二区三区| 国产女人喷潮视频免费| 热の综合热の国产热の潮在线| 自拍偷自拍亚洲精品牛影院| 性开放的女人aaa片| 国产高清视频一区三区| 无码精品国产一区二区免费| 亚洲精品伊人久久久大香 | 人妻少妇偷人精品无码| 欧洲亚洲1卡二卡三卡2021| 国产下药迷倒白嫩美女网站| 亚洲 欧美 中文 在线 视频| 欧美艳星nikki激情办公室| 亚洲中文字幕日产乱码在线 | 日韩av片无码一区二区不卡电影| 国产精品第一国产精品| 亚洲欧洲日韩国内高清| 精品熟女少妇av久久免费软件| 日韩一区二区三区高清电影| 国产亚洲精品久久777777| 波多野结衣乱码中文字幕| 亚欧日韩欧美网站在线看| 精品午夜福利无人区乱码一区| 丝袜老师办公室里做好紧好爽| 色偷偷尼玛图亚洲综合| 成人三级无码视频在线观看| 黄a无码片内射无码视频| 日本狂喷奶水在线播放212| 区二区三区国产精华液区别大吗| 久久不见久久见免费影院www| 日韩人妻中文无码一区二区七区| 77777亚洲午夜久久多喷| 国产精品美女久久久久| 国产最新进精品视频| 日本道精品一区二区三区| 夜夜爱夜鲁夜鲁很鲁| 永久黄网站色视频免费| 男女性潮高清免费网站|