Recombinant Human HMBS protein (ab123176)

製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号P08397
    • 生物種Human
    • 配列MGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATAGLQRM GWHNRVGQIL HPEECMYAVG QGALGVEVRA KDQDILDLVG VLHDPETLLR CIAERAFLRH LEGGCSVPVA VHTAMKDGQL YLTGGVWSLD GSDSIQETMQ ATIHVPAQHE DGPEDDPQLV GITARNIPRG PQLAAQNLGI SLANLLLSKG AKNILDVARQ LNDAH
    • 分子量42 kDa including tags
    • 領域1 to 361
    • タグHis tag N-Terminus

特性

Our Abpromise guarantee covers the use of ab123176 in the following tested applications.

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

  • アプリケーション

    Mass Spectrometry

    SDS-PAGE

  • 質量分析
    MALDI-TOF
  • 精製度> 95 % SDS-PAGE.
    ab123176 is purified using conventional chromatography techniques.
  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol, 0.58% Sodium chloride

関連情報

  • 別名
    • 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
  • 機能Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
  • 組織特異性Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
  • パスウェイPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
  • 関連疾患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.
  • 配列類似性Belongs to the HMBS family.
  • 細胞内局在Cytoplasm.
  • Information by UniProt

Recombinant Human HMBS protein 画像

  • 15% SDS-PAGE analysis of ab123176 (3ug)

Recombinant Human HMBS protein (ab123176) 使用論文

ab123176 has not yet been referenced specifically in any publications.

Product Wall

PBGD enzyme product cat# ab123176 is purified as holo (or apo) enzyme.

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