Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      FTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYS MQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEG
    • Amino acids
      526 to 625
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab163431 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • 3-methylcrotonyl-CoA carboxylase 1
    • 3-methylcrotonyl-CoA carboxylase biotin-containing subunit
    • 3-methylcrotonyl-CoA carboxylase, alpha
    • 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
    • DKFZp686B20267
    • FLJ25545
    • MCC-B
    • MCCA
    • MCCase subunit alpha
    • methylcrotonoyl-CoA carboxylase 1 (alpha)
    • methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
    • methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
    see all
  • Relevance
    MCCC1 is the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1 (MCC1 deficiency). MCC1 deficiency is an autosomal recessive disorder of leucine catabolism.
  • Cellular localization
    Mitochondrion matrix.

Images

  • ab163431 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab163431 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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