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.

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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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