Recombinant人HMBS protein (ab114899)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MSGNGNAAATAEENSPKMRVIRVGTRKSQLARIQTDSVVATLKASYPGLQ FEIIAMSTTGDKIPDTALSKIGEKSLFTKELEHALEKNEVDLVVHSLKDL PTVLPPGFTIGAICKRENPHDAVVFHPKFVGKTLETLPEKSVVGTSSLRR AAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFSAIILATAGLQRMGWHN RVGQILHPEECMYAVGQGALGVEVRAKDQDILDLVGVLHDPETLLRCIAE RAFLRHLEGGCSVPVAVHTAMKDGQLYLTGGVWSLDGSDSIQETMQATIH VPAQHEDGPEDDPQLVGITARNIPRGPQLAAQNLGISLANLLLSKGAKNI LDVARQLNDAH
    • 分子量
      66 kDa including tags
    • 氨基酸
      1 to 361

技术指标

Our Abpromise guarantee covers the use of ab114899 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • HEM3_HUMAN
    • HMBS
    • Hydroxymethylbilane synthase
    • PBG D
    • PBG-D
    • PBGD
    • PORC
    • Porphobilinogen deaminase
    • porphyria, acute; Chester type
    • Pre uroporphyrinogen synthase
    • Pre-uroporphyrinogen synthase
    • UPS
    • Uroporphyrinogen I synthase
    • Uroporphyrinogen I synthetase
    see all
  • 功能
    Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
  • 组织特异性
    Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
  • 通路
    Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
  • 疾病相关
    Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
  • 序列相似性
    Belongs to the HMBS family.
  • 细胞定位
    Cytoplasm.
  • Information by UniProt

图片

  • 12.5% SDS-PAGE showing ab114899 at approximately 65.82kDa.
    Stained with Coomassie Blue.

文献

ab114899 has not yet been referenced specifically in any publications.

客户评价及客户问答

Unfortunately, we have not tested the reactivity of this product and therefore we don’t know if it is an intermediate or as a holo (apo-) enzyme. We have only tested this antibody in WB, SDS-PAGE and ELISA.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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