Recombinant Human L2HGDH protein (ab127239)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE
製品の詳細
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製品名
Recombinant Human L2HGDH protein -
精製度
> 90 % SDS-PAGE.
Purified via His tag -
発現系
Escherichia coli -
アクセッション番号
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タンパク質長
Protein fragment -
Animal free
No -
由来
Recombinant -
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生物種
Human -
予測される分子量
17 kDa -
領域
65 to 216 -
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 ab127239 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
SDS-PAGE
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製品の状態
Lyophilized -
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 -20ºC.
Constituents: 0.32% Tris HCl, 0.58% Sodium chloride
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再構成Reconstitute with water to desired concentration.
関連情報
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別名
- 2 hydroxyglutarate dehydrogenase
- Alpha hydroxyglutarate oxidoreductase
- Alpha ketoglutarate reductase
see all -
組織特異性
Widely expressed. Highly expressed in brain, testis and muscle. Expressed to a lower extent in lymphocytes, fibroblasts, keratinocytes, placenta, bladder, small intestine, liver and bone marrow. -
関連疾患
Defects in L2HGDH are the cause of L-2-hydroxyglutaric aciduria (L2HGA) [MIM:236792]. L2HGA is a rare autosomal recessive disorder clinically characterized by mild psychomotor delay in the first years of life, followed by progressive cerebellar ataxia, dysarthria and moderate to severe mental retardation. Diagnosis is based on the presence of an excess of L-2-hydroxyglutaric acid in urine, blood and cerebrospinal fluid. -
配列類似性
Belongs to the L2HGDH family. -
細胞内局在
Mitochondrion. - 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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Datasheet download
参考文献 (0)
ab127239 は論文での使用が確認できていません。