Overview

  • Product nameAnti-MCCC2 antibody
    See all MCCC2 primary antibodies
  • Description
    Rabbit polyclonal to MCCC2
  • Tested applicationsSuitable for: WB, ELISAmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    A KLH conjugated synthetic peptide selected from the Center region of Human MCCC2.

  • Positive control
    • 293 cell line lysates and mouse liver tissue lysates

Properties

Applications

Our Abpromise guarantee covers the use of ab91414 in the following tested applications.

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

Application Abreviews Notes
WB
ELISA
  • Application notesELISA: 1/1000.
    WB: 1/100 - 1/500. Predicted molecular weight: 61 kDa.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • PathwayAmino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
    • Involvement in diseaseDefects 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 similaritiesBelongs to the AccD/PCCB family.
      Contains 1 carboxyltransferase domain.
    • Cellular localizationMitochondrion matrix.
    • Information by UniProt
    • Database links
    • Alternative names
      • 3 methylcrotonyl CoA carboxylase 2 antibody
      • 3 methylcrotonyl CoA carboxylase non biotin containing subunit antibody
      • 3 methylcrotonyl CoA:carbon dioxide ligase subunit beta antibody
      • 3-methylcrotonyl-CoA carboxylase 2 antibody
      • 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody
      • 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody
      • Biotin carboxylase antibody
      • MCCase subunit beta antibody
      • MCCB antibody
      • MCCB_HUMAN antibody
      • MCCC 2 antibody
      • Mccc2 antibody
      • Methylcrotonoyl CoA carboxylase 2 (beta) antibody
      • Methylcrotonoyl CoA carboxylase beta chain mitochondrial antibody
      • Methylcrotonoyl Coenzyme A carboxylase 2 (beta) antibody
      • Methylcrotonoyl-CoA carboxylase beta chain antibody
      • mitochondrial antibody
      • Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase antibody
      see all

    Anti-MCCC2 antibody images

    • Anti-MCCC2 antibody (ab91414) at 1/100 dilution + 293 cell line lysates at 35 µg

      Predicted band size : 61 kDa
    • Anti-MCCC2 antibody (ab91414) at 1/100 dilution + mouse liver tissue lysates at 35 µg

      Predicted band size : 61 kDa

    References for Anti-MCCC2 antibody (ab91414)

    ab91414 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"