Overview

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

    Recombinant fragment, corresponding to amino acids 456-564 of Human MCCC2 (NP_071415) with a proprietary tag.

  • Positive control
    • Human liver tissue, MCCC2 transfected 293T cell lysate

Properties

Associated products

Applications

Our Abpromise guarantee covers the use of ab118370 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 Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 61 kDa.
IHC-P Use a concentration of 3 µg/ml. Antigen retrieval is not essential but may optimise staining. Place sample in 1X citrate buffer (pH 6.0) in pressure cooker under 125oC for 4min and under 90oC for 45min.

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

  • All lanes : Anti-MCCC2 antibody (ab118370) at 5 µg/ml

    Lane 1 : MCCC2 transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 61 kDa
  • Formalin-fixed, paraffin-embedded Human liver tissue labelled with ab118370 at 3 µg/ml
  • Anti-MCCC2 antibody (ab118370) at 5 µg/ml + Recombinant Protein (immunogen) at 0.2 µg

    Predicted band size : 61 kDa

References for Anti-MCCC2 antibody (ab118370)

ab118370 has not yet been referenced specifically in any publications.

Product Wall

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