Recombinant人HMGCS2 protein (ab114900)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP54868
    • 种属Human
    • 序列MQRLLTPVKRILQLTRAVQETSLTPARLLPVAHQRFSTASAVPLAKTDTW PKDVGILALEVYFPAQYVDQTDLEKYNNVEAGKYTVGLGQTRMGFCSVQE DINSLCLTVVQRLMERIQLPWDSVGRLEVGTETIIDKSKAVKTVLMELFQ DSGNTDIEGIDTTNACYGGTASLFNAANWMESSSWDGRYAMVVCGDIAVY PSGNARPTGGAGAVAMLIGPKAPLALERGLRGTHMENVYDFYKPNLASEY PIVDGKLSIQCYLRALDRCYTSYRKKIQNQWKQAGSDRPFTLDDLQYMIF HTPFCKMVQKSLARLMFNDFLSASSDTQTSLYKGLEAFGGLKLEDTYTNK DLDKALLKASQDMFDKKTKASLYLSTHNGNMYTSSLYGCLASLLSHHSAQ ELAGSRIGAFSYGSGLAASFFSFRVSQDAAPGSPLDKLVSSTSDLPKRLA SRKCVSPEEFTEIMNQREQFYHKVNFSPPGDTNSLFPGTWYLERVDEQHR RKYARRPV
    • 分子量82 kDa including tags
    • 氨基酸1 to 508

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技术指标

Our Abpromise guarantee covers the use of ab114900 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
    • 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
  • 功能This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
  • 组织特异性High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
  • 通路Metabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3.
  • 疾病相关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.
  • 序列相似性Belongs to the HMG-CoA synthase family.
  • 细胞定位Mitochondrion.
  • Information by UniProt

Recombinant Human HMGCS2 protein 图像

  • 12.5% SDS-PAGE showing ab114900 at approximately 81.95kDa.
    Stained with Coomassie Blue.

Recombinant Human HMGCS2 protein (ab114900)参考文献

ab114900 has not yet been referenced specifically in any publications.

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