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, FITC conjugated

  • 中文名稱:
    NSDHL兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA618994LC01HU
  • 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:
    FITC
  • 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
  • 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: 在线精品无码字幕无码av| 国产一区二区在线视频| 亚洲卡1卡2卡新区网站| 亚洲日韩成人性av网站| 国内外精品激情刺激在线 | 国产a∨精品一区二区三区不卡 | 国产97在线 | 中文| 亚洲欧美国产另类va| 日本老熟妇乱子伦视频| 欧美又黄又大又爽a片三年片| 欧美性黑人极品hd| 99久久精品国产一区二区蜜芽 | 夜夜爽妓女8888视频免费观看 | 国产精品久久久久9999赢消| 丰满人妻一区二区三区无码av | 国产精品99久久不卡| 亚洲 欧美 日韩 综合aⅴ电影| 国产午夜高清高清在线观看| 五月天激情国产综合婷婷婷| 久久精品国产99国产精品导航| 最新版天堂资源中文官网| 国产精品香蕉视频在线| 久久婷婷五月综合97色| 国产成人久久精品流白浆| 国产成人一区二区三区在线观看| 久久99日韩国产精品久久99| 色诱久久久久综合网ywww| 国产午夜亚洲精品不卡在线观看| 中文乱码免费一区二区| 欧美黑人巨大videos极品| 乱中年女人伦av三区| 自拍日韩亚洲一区在线| 亚洲综合区小说区激情区| 国产成人愉拍精品| 一区二区三区无码被窝影院| 亚洲精品久久久无码av片软件| 久久无码精品一一区二区三区| 最新av偷拍av偷窥av网站| 国产精品自在线拍国产| 国产精品亚洲а∨天堂| 亚洲人成网线在线播放va蜜芽|