merck millipore,默克密理愽
  • merck millipore,默克密理博,ABN366,Anti-Porphobilinogen Deaminase Antibody

  • merck millipore,默克密理博,ABN366,Anti-Porphobilinogen Deaminase Antibody

    产品名称：Anti-Porphobilinogen Deaminase Antibody
    产品型号：ABN366
    Detect Porphobilinogen deaminase using this rabbit polyclonal antibody, Anti-Porphobilinogen Deaminase Antibody validated for use in western blotting & IHC (Paraffin).

    merck millipore,默克密理博,ABN366,Anti-Porphobilinogen Deaminase Antibody

  • 产品介绍
  • merck millipore,默克密理博,ABN366,Anti-Porphobilinogen Deaminase Antibody

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    R, HWBRbAffinity PurifiedPolyclonal Antibody
    描述
    产品目录编号 ABN366
    描述 Anti-Porphobilinogen Deaminase Antibody
    Alternate Names
    • Pre-uroporphyrinogen synthase
    • Porphobilinogen deaminase
    • PBG-D
    • Hydroxymethylbilane synthase
    • HMBS
    背景信息 The protein named Porphobilinogen deaminase (PBGD), or alternatively, Hydroxymethylbilane synthase (HMBS), or Pre-uroporphyrinogen synthase, is a key synthetic enzyme in Heme synthesis. Heme is the pigment in red blood cells and is a cofactor of the protein hemoglobin. This enzyme catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in PBGD are associated with acute intermittent porphyria (AIP). Interestingly, in recent studies of human women subjects that suffer from AIP also demonstrated marked upset in a variety of biological-clock controlled gene mRNAs which may trigger or sustain some of the common clinical features associated with AIP. Moreover in knockout mice, Porphobilinogen deaminase double knockouts develop impairment of motor coordination and muscle weakness and demonstrate ultrastructural changes consistent with primary motor axon degeneration, secondary Schwann cell reactions, and axonal regeneration and suggest that heme deficiency and consequent dysfunction of hemeproteins can cause porphyric neuropathy.
    产品信息
    格式 Affinity Purified
    演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
    应用
    应用 Detect Porphobilinogen deaminase using this rabbit polyclonal antibody, Anti-Porphobilinogen Deaminase Antibody validated for use in western blotting & IHC (Paraffin).
    主要应用
    • Western Blotting
    应用说明 Immunohistochemistry Analysis: A 1:2,000 dilution from a representative lot detected Porphobilinogen Deaminase in human hyperplastic prostate tissue.
    生物信息
    免疫原品种 KLH-conjugated linear peptide corresponding to the C-Terminus of human Porphobilinogen Deaminase.
    浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration.
    宿主 Rabbit
    特异性 This antibody is expected to cross react with four known isoforms based upon immunogen design.
    品种反应性 RatHuman
    Species Reactivity Note Demonstrated to react with Human and Rat. Predicted to react with Rhesus Macaque based on 100% sequence homology.
    抗体类型 Polyclonal Antibody
    Entrez基因编号
    • NP_000181
    基因符号
    • UPS
    • HMBS
    • PBGD
    纯化方法 Affinity purified
    UniProt编号
    • P08397
    产品使用声明
    质量保证 Evaluated by Western Blotting in K562 cell lysate.

    Western Blotting Analysis: 0.1 µg/mL of this antibody detected Porphobilinogen Deaminase in 10 µg of K562 cell lysate.
    使用声明
    • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
    储存和货运信息
    存储条件 Stable for 1 year at 2-8°C from date of receipt.
    包装信息
    数量 100 µg

    merck millipore,默克密理博,ABN366,Anti-Porphobilinogen Deaminase Antibody

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