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

NPHP3 Antibody

  • 中文名稱:
    NPHP3兔多克隆抗體
  • 貨號(hào):
    CSB-PA768767ESR1HU
  • 規(guī)格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: NPHP3 antibody at 1.57 μg/ml + Mouse gonadal tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 151, 74, 72, 20, 27, 80, 16 kDa
      Observed band size: 151 kDa
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱:
    Rabbit anti-Homo sapiens (Human) NPHP3 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    NPHP3
  • 別名:
    Meckel syndrome, type 7 antibody; MKS7 antibody; Nephrocystin-3 antibody; nephronophthisis 3 (adolescent) antibody; NPH3 antibody; Nphp3 antibody; NPHP3_HUMAN antibody; pcy antibody; RHPD antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Nephrocystin-3 protein (1-130AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Non-conjugated
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity Purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產(chǎn)品提供形式:
    Liquid
  • 應(yīng)用范圍:
    ELISA, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:1000-1:5000
  • Protocols:
  • 儲(chǔ)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(píng)價(jià)

靶點(diǎn)詳情

  • 功能:
    Required for normal ciliary development and function. Inhibits disheveled-1-induced canonical Wnt-signaling activity and may also play a role in the control of non-canonical Wnt signaling which regulates planar cell polarity. Probably acts as a molecular switch between different Wnt signaling pathways. Required for proper convergent extension cell movements.
  • 基因功能參考文獻(xiàn):
    1. Case Report: NPHP3 related nephronophthisis manifesting in the fetal period. PMID: 28921755
    2. Inherited 3 deleterious mutations in two nephronophthisis genes, NPHP3 and NPHP4 cause unusually severe form of infantile nephronophthisis. PMID: 28392475
    3. NPHP3 mutations were prevalent in Chinese infantile nephronophthisis patients. All patients with NPHP3 mutations showed renal-hepatic phenotype. PMID: 26184788
    4. a rare neonatal ciliopathy presentation of NPHP3 mutations leading to severe multiorgan failure in two siblings. PMID: 24776604
    5. The known phenotype of NPHP3 mutation caused renal-hepatic-pancreatic dysplasia has been extended to include skeletal and CNS anomalies. PMID: 23686967
    6. ANKS6 as a new NPHP family member that assembles a distinct module of nephronophthisis-associated proteins, encompassing NEK8, INVS and NPHP3. PMID: 23793029
    7. Mutations in a novel gene, NPHP3, cause adolescent nephronophthisis, tapeto-retinal degeneration and hepatic fibrosis. PMID: 12872122
    8. In six families with nephronophthisis, there were two mutations in either NPHP1, NPHP3, or NPHP4, suggesting oligogenicity. PMID: 17855640
    9. NPHP3 mutations can cause a broad clinical spectrum of early embryonic patterning defects. PMID: 18371931
    10. screened 43 families with infantile nephronophthisis (ESRD less than 5 years of age) for NPHP2 and NPHP3 mutations and determined genotype-phenotype correlations PMID: 19177160
    11. The presence of congenital malformations in the case series confirms the crucial role of NPHP3 in early embryonic development of the kidneys and urinary tract. PMID: 19303681

    顯示更多

    收起更多

  • 相關(guān)疾?。?/div>
    Nephronophthisis 3 (NPHP3); Renal-hepatic-pancreatic dysplasia 1 (RHPD1); Meckel syndrome 7 (MKS7)
  • 亞細(xì)胞定位:
    Cell projection, cilium. Note=Localization to cilium is mediated via interaction with UNC119 and UNC119B, which bind to the myristoyl moiety of the N-terminus.
  • 組織特異性:
    Widely expressed at low level. Expressed in heart, placenta, liver, skeletal muscle, kidney and pancreas. Expressed at very low level in brain and lung.
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 7907

    OMIM: 208540

    KEGG: hsa:27031

    STRING: 9606.ENSP00000338766

    UniGene: Hs.511991



主站蜘蛛池模板: 久久久久蜜桃精品成人片公司 | 亚洲欧美中文字幕日韩一区二区 | 中国少妇初尝黑人巨高清| 国产一区二区精品丝袜| 欧美性欧美巨大黑白大战| 天堂8а√中文在线官网| 国产成人精品手机在线观看| 黄a大片av永久免费| 日韩人妻无码一区二区三区综合部| 国产高潮又爽又刺激的视频| 精品多人p群无码| 一区二区三区国产| 精品人妻系列无码人妻不卡| 无码专区―va亚洲v专区在线 | 妺妺窝人体色www在线观看| 亚洲日韩中文字幕一区| 亚洲欧美韩国综合色| 亚洲乱码卡一卡二卡新区仙踪木木 | 波多野结衣爽到高潮大喷| 国产又色又爽又黄的在线观看| 久久精品国产精品亚洲艾草网| 白嫩日本少妇做爰| 国产一区二区三区在线视頻| 狠狠噜天天噜日日噜视频麻豆| 好爽别插了无码视频| 免费无码一区二区三区蜜桃大 | 精品久久久久久无码中文野结衣| 免费无码av片在线观看动漫| 免费精品国偷自产在线2020| 97高清国语自产拍| 中文字幕av在线一二三区| 国产伦人人人人人人性| 亚洲日韩欧美在线成人| 粗大猛烈进出高潮视频免费看| 精品自拍亚洲一区在线| 波多野结衣一区二区免费视频| 日本少妇毛茸茸高潮| 成人国产三级在线观看| 成人免费网站视频www| 国产精品成人无码免费| 成人做爰视频www网站小优视频|