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DNAH5 Antibody, HRP conjugated

  • 中文名稱:
    DNAH5兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA819476LB01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) DNAH5 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    DNAH5
  • 別名:
    axonemal antibody; Axonemal beta dynein heavy chain 5 antibody; Ciliary dynein heavy chain 5 antibody; DNAH 5 antibody; Dnah5 antibody; DNAHC5 antibody; DYH5_HUMAN antibody; Dynein heavy chain 5 antibody; Dynein heavy chain 5; axonemal antibody; HL1 antibody; KIAA1603 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Dynein heavy chain 5, axonemal protein (2364-2560AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    HRP
  • 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:
    Force generating protein of respiratory cilia. Produces force towards the minus ends of microtubules. Dynein has ATPase activity; the force-producing power stroke is thought to occur on release of ADP. Required for structural and functional integrity of the cilia of ependymal cells lining the brain ventricles.
  • 基因功能參考文獻:
    1. A novel mutation causing primary ciliary dyskinesia was found in Japanese patients. PMID: 28939216
    2. to the best of our knowledge, the first reported case of PCD caused by the DNAH5 mutation in a Japanese patient. PMID: 27779714
    3. These findings demonstrated that new DNAH5 mutations could be used for molecular diagnosis of Kartagener syndrome, providing families with genetic counseling and prenatal diagnosis. PMID: 27988889
    4. The mutation of p.Glu2610Gly in DNAH5 is novel. PMID: 24912412
    5. In chronic obstructive pulmonary disease, total lung capacity was associated with a SNP in DNAH5. PMID: 25134640
    6. A novel mutation in DNAH5 (c. 8030G>A) is identified in a Han Chinese family with chronic rhinosinusitis and primary ciliary dyskinesia. PMID: 24150548
    7. DNAH5 (rs2277046) is associated with single nucleotide polymorphisms in Korean patients, either non-segmental or segmental type. PMID: 23678272
    8. DNAH5 is the cause of primary ciliary dyskinesia in two siblings in a nuclear family as identified by whole genome sequencing. PMID: 20220176
    9. immunofluorescence studies of respiratory epithelial cells with secondary ciliary dyskinesia identified in vitro and in vivo normal axonemal DNAH5 localization PMID: 16492982
    10. DNAH5 is frequently mutated in patients with primary ciliary dyskinesia exhibiting outer dynein arm defects and mutations cluster in five exons. PMID: 16627867
    11. Male carriers of the mutations always exhibit asthenozoospermia, whereas female carriers manifest no alterations in either fertility or pulmonary clearance. PMID: 18492703
    12. Two dynein genes, encoding ODA intermediate chain (DNAI1) and heavy chain (DNAH5), have been seen to be mutated in approximately 30-38% of the families PMID: 19300264
    13. Although genetic alterations remain to be identified in most patients, DNAH5 is to date the main PCD gene. PMID: 19357118
    14. Two "major" genes, DNAI1 and DNAH5, underlie PCD in nearly half of the patients with ODA defects PMID: 19410201

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  • 相關疾病:
    Ciliary dyskinesia, primary, 3 (CILD3)
  • 亞細胞定位:
    Cytoplasm, cytoskeleton, cilium axoneme.
  • 蛋白家族:
    Dynein heavy chain family
  • 組織特異性:
    Expressed in airway epithelial cells (at protein level). Not detected in spermatozoa (at protein level).
  • 數據庫鏈接:

    HGNC: 2950

    OMIM: 603335

    KEGG: hsa:1767

    STRING: 9606.ENSP00000265104

    UniGene: Hs.212360



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