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: 亚洲国产精品久久一线不卡| 精品视频国产狼友视频| 国产亚洲日本精品无码| 男女啪啦猛视频免费| 日本高清一区二区三| 236宅宅理论片免费| 成在人av抽搐高潮喷水流白浆| 五月丁香综合缴情六月小说| 亚洲欧美日韩国产成人一区| 人体内射精一区二区三区| 久久精品麻豆日日躁夜夜躁| 无码人妻aⅴ一区二区三区有奶水| 亚洲日韩一区二区一无码| 免费啪视频在线观看视频日本| 亚洲综合中文字幕无线码| 久久人人爽人人爽人人片av不 | 欧美成人精品第一区二区三区| 亚洲熟妇中文字幕日产无码| 伊人久久大香线蕉综合5g| 中文天堂最新版资源www官网| 欧美成人一区二区三区片免费| 久久久亚洲欧洲日产国码农村 | 国产精品vr专区| 亚洲精品欧美综合四区| 青青草99久久精品国产综合| 亚洲性啪啪无码av天堂| 久久综合狠狠色综合伊人| 秋霞最新高清无码鲁丝片| 国产精品视频色拍在线视频| 亚洲肥老太bbw中国熟女| 午夜精品久久久久久中宇| 国产真实伦在线观看视频| 潮喷无码正在播放| 精品国偷自产在线| 久久亚洲美女精品国产精品| 国产 成人 综合 亚洲 网站| 西西人体444www高清大胆| 日产中文字幕在线观看| 亚洲乱亚洲乱妇中文影视| 欧美精品a∨在线观看| 三级特黄60分钟在线播放|