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

NSDHL Antibody, Biotin conjugated

  • 中文名稱:
    NSDHL兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA618994LD01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) NSDHL Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    NSDHL
  • 別名:
    NSDHL; H105E3; Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating; Protein H105e3
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Sterol-4-alpha-carboxylate 3-dehydrogenase, decarboxylating protein (154-211AA)
  • 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:
    Catalyzes the NAD(P)(+)-dependent oxidative decarboxylation of the C4 methyl groups of 4-alpha-carboxysterols in post-squalene cholesterol biosynthesis. Plays also a role in the regulation of the endocytic trafficking of EGFR.
  • 基因功能參考文獻:
    1. Here we present the case of a 9-year-old Chinese girl born with the typical clinical features of CHILD syndrome. Evaluation of the skin lesions confirmed the diagnosis and led to identification of a heterozygous point mutation in exon 8 of the NSDHL gene. PMID: 26459993
    2. Our findings expand the spectrum of mutations in NSDHL in CHILD syndrome, and indicate that large exon deletions may be not rare. PMID: 26014843
    3. A novel missense mutation in the NSDHL gene identified in a Lithuanian family by targeted next-generation sequencing causes CK syndrome. PMID: 25900314
    4. human NSDHL protein and mouse Nsdhl mRNA were expressed in tissues synthesizing cholesterol and steroids and in all peripheral tissues affected by CHILD or CK syndromes. PMID: 22113624
    5. found that males with intellectual disability in another reported family with an NSDHL mutation (c.1098 dup [p.Arg367SerfsX33]) have CKS PMID: 21129721
    6. The missense mutation of the NSDHL gene is detected in CHILD syndrome. PMID: 19906044
    7. Lethality of Nsdhl deficient mouse embryos is rescued by transgenic mice expressing human Nsdhl. PMID: 19880419
    8. NAD(P)H steroid dehydrogenase-like protein is localized to lipid droplets PMID: 12837764
    9. NSDHL, an enzyme involved in cholesterol synthesis, traffics through the Golgi and accumulates on ER membranes and on the surface of lipid droplets. PMID: 14506130
    10. microarray analysis of gene expression related to NSDHL sterol dehydrogenase in embryonic fibroblasts PMID: 15805545
    11. A novel missense mutation (R199H) in exon 6 of the NSDHL gene was identified in a small subset of sporadic verruciform xanthomas. PMID: 16230564
    12. NAD(P) dependent steroid dehydrogenase-like (NSDHL)-shRNA sequences were designed and tested for their effectiveness. PMID: 17498944

    顯示更多

    收起更多

  • 相關疾病:
    Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD); CK syndrome (CKS)
  • 亞細胞定位:
    Endoplasmic reticulum membrane; Single-pass membrane protein. Lipid droplet.
  • 蛋白家族:
    3-beta-HSD family
  • 組織特異性:
    Brain, heart, liver, lung, kidney, skin and placenta.
  • 數據庫鏈接:

    HGNC: 13398

    OMIM: 300275

    KEGG: hsa:50814

    STRING: 9606.ENSP00000359297

    UniGene: Hs.57698



主站蜘蛛池模板: 强开小婷嫩苞又嫩又紧韩国视频| 高清av熟女一区| 国产精品成人观看视频| 国产人妻精品一区二区三首 | 色一情一区二| 学生精品国产在线视频| 日本一卡2卡3卡4卡5卡精品视频| 人妻中文乱码在线网站| 亚洲人成网站色7799| 色婷婷av久久久久久久| 国产一区二区在线视频| 风韵人妻丰满熟妇老熟女| 欧美人与动zozo在线播放| 西西人体午夜大胆无码视频| 国产免费又色又爽粗视频| 欧美性猛交xxxx免费视频软件| 午夜福利麻豆国产精品| 亚洲综合色在线观看一区| 日本特黄aaaaaa大片| 男人j进入女人j内部免费网站| 欧美日韩国产精品自在自线| 国内熟女啪啪自拍| 精品国产卡一卡2卡3卡| 野花社区视频www官网| 大学生高潮无套内谢视频| 亚洲精品福利一区二区三区蜜桃| 中文字幕久久精品一区二区三区 | 欧美性猛交xxxx富婆| 琪琪秋霞午夜av影视在线| 国产福利在线永久视频| 欧美黑人大战白嫩在线| 成人性能视频在线| 久久综合激激的五月天| 动漫无遮挡羞视频在线观看| 国产在线观看免费人成视频| 欧美乱妇高清无乱码| 亚洲乱码中文字幕久久孕妇黑人| 亚洲成a人v在线蜜臀| 国产精品日本亚洲欧美| 国模吧无码一区二区三区| 久久久久久久无码高潮|