Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MWAVLRLALRPCARASPAGPRAYHGDSVASLGTQPDLGSALYQENYKQMK ALVNQLHERVEHIKLGGGEKARALHISRGKLLPRERIDNLIDPGSPFLEL SQFAGYQLYDNEEVPGGGIITGIGRVSGVECMIIANDATVKGGAYYPVTV KKQLRAQEIAMQNRLPCIYLVDSGGAYLPRQADVFPDRDHFGRTFYNQAI MSSKNIAQIAVVMGSCTAGGAYVPAMADENIIVRKQGTIFLAGPPLVKAA TGEEVSAEDLGGADLHCRKSGVSDHWALDDHHALHLTRKVVRNLNYQKKL DVTIEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFTEFKAF YGDTLVTGFARIFGYPVGIVGNNGVLFSESAKKGTHFVQLCCQRNIPLLF LQNITGFMVGREYEAEGIAKDGAKMVAAVACAQVPKITLIIGGSYGAGNY GMCGRAYSPRFLYIWPNARISVMGGEQAANVLATITKDQRAREGKQFSSA DEAALKEPIIKKFEEEGNPYYSSARVWDDGIIDPADTRLVLGLSFSAALN APIEKTDFGIFRM
    • Amino acids
      1 to 563
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab163770 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 2
    • 3 methylcrotonyl CoA carboxylase non biotin containing subunit
    • 3 methylcrotonyl CoA:carbon dioxide ligase subunit beta
    • 3-methylcrotonyl-CoA carboxylase 2
    • 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit
    • 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
    • Biotin carboxylase
    • MCCase subunit beta
    • MCCB
    • MCCB_HUMAN
    • MCCC 2
    • Mccc2
    • Methylcrotonoyl CoA carboxylase 2 (beta)
    • Methylcrotonoyl CoA carboxylase beta chain mitochondrial
    • Methylcrotonoyl Coenzyme A carboxylase 2 (beta)
    • Methylcrotonoyl-CoA carboxylase beta chain
    • mitochondrial
    • Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase
    see all
  • Pathway
    Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
  • Involvement in disease
    Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) [MIM:210210]. MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.
  • Sequence similarities
    Belongs to the AccD/PCCB family.
    Contains 1 carboxyltransferase domain.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt

Images

  • ab163770 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab163770 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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