Recombinant人HMBS protein (ab114899)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP08397
    • 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

Recombinant Human HMBS protein 图像

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

Recombinant Human HMBS protein (ab114899)参考文献

ab114899 has not yet been referenced specifically in any publications.

Product Wall

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"