MCCC2 Antibody

Code CSB-PA867199ESR1HU
Size US$166
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  • Western blot
    All lanes: MCCC2 antibody at 6.85µg/ml
    Lane 1: Mouse brain tissue
    Lane 2: Hela whole cell lysate
    Lane 3: MCF-7 whole cell lysate
    Lane 4: K562 whole cell lysate
    Lane 5: HepG2 whole cell lysate
    Lane 6: A549 whole cell lysate
    Secondary
    Goat polyclonal to rabbit IgG at 1/10000 dilution
    Predicted band size: 62, 58 kDa
    Observed band size: 62 kDa

  • Immunohistochemistry of paraffin-embedded human pancreatic cancer using CSB-PA867199ESR1HU at dilution of 1:100

  • Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA867199ESR1HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) MCCC2 Polyclonal antibody
Uniprot No.
Target Names
MCCC2
Alternative Names
MCCC2 antibody; MCCBMethylcrotonoyl-CoA carboxylase beta chain antibody; mitochondrial antibody; MCCase subunit beta antibody; EC 6.4.1.4 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
Raised in
Rabbit
Species Reactivity
Human, Mouse
Immunogen
Recombinant Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Polyclonal
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:1000-1:5000
IHC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Gene References into Functions
  1. Novel mutation in MCCC2 gene was identified in Chinese population. PMID: 25382614
  2. Mutation in 3-methylcrotonyl CoA carboxylase 2 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
  3. The amino-termini containing 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
  4. The Kd value of soraphen A for the BC domains of human ACC1 and ACC2 is 1 nM. This high binding affinity is mainly due to the extensive interactions between soraphen A and the human biotin carboxylase domain PMID: 17876819
Involvement in disease
3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
Subcellular Location
Mitochondrion matrix.
Protein Families
AccD/PCCB family
Database Links

HGNC: 6937

OMIM: 210210

KEGG: hsa:64087

STRING: 9606.ENSP00000343657

UniGene: Hs.604789

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