Recombinant Human HMGCS2 protein (ab114901)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP54868
    • SpeciesHuman
    • SequenceRVSQDAAPGSPLDKLVSSTSDLPKRLASRKCVSPEEFTEIMNQREQFYHK VNFSPPGDTNSLFPGTWYLERVDEQHRRKYARRPV
    • Molecular weight35 kDa including tags
    • Amino acids424 to 508

Associated products

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • FormLiquid
  • Additional notesProtein 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
  • FunctionThis enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase.
  • Tissue specificityHigh expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney.
  • PathwayMetabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the HMG-CoA synthase family.
  • Cellular localizationMitochondrion.
  • Information by UniProt

Recombinant Human HMGCS2 protein images

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

References for Recombinant Human HMGCS2 protein (ab114901)

ab114901 has not yet been referenced specifically in any publications.

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