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: 婷婷久久香蕉五月综合| 欧美日韩精品一区二区在线播放| 精品国产av色一区二区深夜久久| 精品国产乱码久久久久app下载| 国产精品亚洲专区无码老司国 | 又粗又猛又黄又爽无遮挡| 人妻丰满熟妇av无码片| 美女又大又黄www免费网站| 久久人人爽人人爽人人片av高清| 四虎成人精品国产永久免费无码| 2021国产精品视频网站| 麻豆精品偷拍人妻在线网址| 亚洲国产精品一区二区www| 欧美丰满老熟妇xxxxx性| 粉嫩虎白女毛片人体| 女上男下啪啪激烈高潮无遮盖| 国产精品久久久久久久久鸭| 性色做爰片在线观看ww | 国产专区国产av| 亚洲不乱码卡一卡二卡4卡5| 无码av岛国片在线播放| 无码伊人66久久大杳蕉网站谷歌| 夜夜添无码一区二区三区| 国产乱人伦偷精品视频免下载| 伊人无码一区二区三区| 巨乳美乳一区二区三区| 精品国产偷窥一区二区| 国产麻豆 9l 精品三级站| 2018高清日本一道国产-在| 色综合久久久久久久久五月| 久久婷婷色综合一区二区| 日韩精品无码一区二区忘忧草| 少妇极品熟妇人妻200片| 美女视频黄频a免费| 一二三区乱码不卡手机版| 免费看撕开奶罩揉吮奶头视频| 亚洲图片综合图区20p| 国产乱子夫妻xx黑人xyx真爽| 69天堂人成无码免费视频| 特级a欧美做爰片第一次| 中国女人做爰视频|