Recombinant Human HMBS/PBGD protein (ab123176)
Key features and details
- Expression system: Escherichia coli
- Purity: > 95% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: MS, SDS-PAGE
製品の詳細
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製品名
Recombinant Human HMBS/PBGD protein -
精製度
> 95 % SDS-PAGE.
ab123176 is purified using conventional chromatography techniques. -
発現系
Escherichia coli -
アクセッション番号
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タンパク質長
Full length protein -
Animal free
No -
由来
Recombinant -
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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 -
予測される分子量
42 kDa including tags -
領域
1 to 361 -
タグ
His tag N-Terminus
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関連製品
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Related Products
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.
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アプリケーション
Mass Spectrometry
SDS-PAGE
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質量分析
MALDI-TOF -
製品の状態
Liquid -
備考
This product was previously labelled as HMBS
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Concentration information loading...
前処理および保存
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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 (glycerin, glycerine), 0.58% Sodium chloride
関連情報
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別名
- HEM3_HUMAN
- HMBS
- Hydroxymethylbilane synthase
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
プロトコール
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (0)
ab123176 は論文での使用が確認できていません。