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NSDHL Recombinant Monoclonal Antibody

  • 中文名稱：
    NSDHL Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA796631A0HU
  • 規格：
    ￥1320
  • 圖片：
    • Flow cytometric analysis of NSDHL expression in HT-1080 cells using NSDHL antibody. Green, isotype control; red, NSDHL.
    • Immunocytochemical staining of HT-1080 cells with NSDHL antibody. Nuclei were stained blue with DAPI; NSDHL was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Medium. Scale bar, 20 μm.
    • Western blotting analysis using NSDHL antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with NSDHL antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    NSDHL
  • 別名：
    NAD(P) Dependent Steroid Dehydrogenase-Like; SDR31E1; XAP104; H105e3; Sterol-4-Alpha-Carboxylate 3-Dehydrogenase, Decarboxylating; Short Chain Dehydrogenase/Reductase Family 31E, Member 1; Protein H105e3; EC 1.1.1.170; NAD(P) Dependent Steroid Dehydrogenase-Like Protein Transcript; Epididymis Secretory Sperm Binding Protein; H105E3
  • 反應種屬：
    Human, Mouse, Rat
  • 免疫原：
    Recombinant Human NSDHL protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    1E2
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, FC, ICC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    FC 1:200-1:2000
    ICC 1:100-1:1000
  • 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

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  • 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