Recombinant Human HMGCS2 protein (ab114901)

製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P54868
    • 生物種Human
    • 配列RVSQDAAPGSPLDKLVSSTSDLPKRLASRKCVSPEEFTEIMNQREQFYHK VNFSPPGDTNSLFPGTWYLERVDEQHRRKYARRPV
    • 分子量35 kDa including tags
    • 領域424 to 508

関連製品

特性

Our Abpromise guarantee covers the use of ab114901 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

    Western blot

    SDS-PAGE

    ELISA

  • 製品の状態Liquid
  • 備考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...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 3 hydroxy 3 methylglutaryl coenzyme A synthase
    • 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2
    • 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial)
    • 3-hydroxy-3-methylglutaryl coenzyme A synthase
    • HMCS2_HUMAN
    • HMG CoA synthase
    • HMG-CoA synthase
    • HMGCS 2
    • HMGCS2
    • Hydroxymethylglutaryl CoA synthase
    • Hydroxymethylglutaryl CoA synthase mitochondrial
    • Hydroxymethylglutaryl-CoA synthase
    • mitochondrial
    see all
  • 機能This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
  • 組織特異性High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
  • パスウェイMetabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3.
  • 関連疾患Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency) [MIM:605911]; also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids.
  • 配列類似性Belongs to the HMG-CoA synthase family.
  • 細胞内局在Mitochondrion.
  • Information by UniProt

Recombinant Human HMGCS2 protein 画像

  • 12.5% SDS-PAGE showing ab114901 at approximately 34.98kDa.
    Stained with Coomassie Blue.

Recombinant Human HMGCS2 protein (ab114901) 使用論文

ab114901 has not yet been referenced specifically in any publications.

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