Overview

  • Product name

    Recombinant Human HMGCS2 protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MQRLLTPVKRILQLTRAVQETSLTPARLLPVAHQRFSTASAVPLAKTDTW PKDVGILALEVYFPAQYVDQTDLEKYNNVEAGKYTVGLGQTRMGFCSVQE DINSLCLTVVQRLMERIQLPWDSVGRLEVGTETIIDKSKAVKTVLMELFQ DSGNTDIEGIDTTNACYGGTASLFNAANWMESSSWDGRYAMVVCGDIAVY PSGNARPTGGAGAVAMLIGPKAPLALERGLRGTHMENVYDFYKPNLASEY PIVDGKLSIQCYLRALDRCYTSYRKKIQNQWKQAGSDRPFTLDDLQYMIF HTPFCKMVQKSLARLMFNDFLSASSDTQTSLYKGLEAFGGLKLEDTYTNK DLDKALLKASQDMFDKKTKASLYLSTHNGNMYTSSLYGCLASLLSHHSAQ ELAGSRIGAFSYGSGLAASFFSFRVSQDAAPGSPLDKLVSSTSDLPKRLA SRKCVSPEEFTEIMNQREQFYHKVNFSPPGDTNSLFPGTWYLERVDEQHR RKYARRPV
    • Molecular weight

      82 kDa including tags
    • Amino acids

      1 to 508

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form

    Liquid
  • Additional notes

    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...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names

    • 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
  • Function

    This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
  • Tissue specificity

    High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
  • Pathway

    Metabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the HMG-CoA synthase family.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt

Images

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

References

ab114900 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab114900.
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