MCCC1 Antibody

Code CSB-PA853497EA01HU
Size US$166
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  • Immunofluorescence staining of A549 cells with CSB-PA853497EA01HU at 1:166, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) MCCC1 Polyclonal antibody
Uniprot No.
Target Names
Alternative Names
MCCC1 antibody; MCCAMethylcrotonoyl-CoA carboxylase subunit alpha antibody; mitochondrial antibody; MCCase subunit alpha antibody; EC antibody; 3-methylcrotonyl-CoA carboxylase 1 antibody; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit antibody; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha antibody
Raised in
Species Reactivity
Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (1-200AA)
Immunogen Species
Homo sapiens (Human)

The MCCC1 Antibody (Product code: CSB-PA853497EA01HU) is Non-conjugated. For MCCC1 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA853497EB01HU MCCC1 Antibody, HRP conjugated ELISA
FITC CSB-PA853497EC01HU MCCC1 Antibody, FITC conjugated
Biotin CSB-PA853497ED01HU MCCC1 Antibody, Biotin conjugated ELISA
Purification Method
>95%, Protein G purified
It differs from different batches. Please contact us to confirm it.
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Tested Applications
Recommended Dilution
Application Recommended Dilution
IF 1:50-1:200
Troubleshooting and FAQs
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

Biotin-attachment 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. MCCC1 plays an essential role in virus-triggered, MAVS-mediated activation of NF-kappaB signaling. PMID: 27629939
  2. This study reports data mainly obtained from the Portuguese newborn screening program collected over a ten-year period. Analysis of the MCCC1 and MCCC2 genes yielded 26 previously unreported mutations and a variant of clinically unknown significance. PMID: 27601257
  3. Our study provides strong support for the susceptibility role of RAB7L1/NUCKS1 rs823118 and MCCC1 rs12637471 in sporadic Parkinson's disease in a Han Chinese population PMID: 26914237
  4. Novel mutations in MCCC1 gene were identified in Chinese population. The expression profiles of two splice mutations (c.639+2T>A and c.639+5G>T) were also characterized. PMID: 25382614
  5. Four new point mutations were detected in the MCCC1 gene in patients with maternal 3-methylcrotonyl coenzyme deficiency. PMID: 24078573
  6. This study demonistrated that Mainland China demonstrates that MCCC1/LAMP3 (rs11711441) is associated with a lower risk of Parkinson's disease. PMID: 23496138
  7. 3-methylcrotonyl-CoA carboxylase inhibition has a role in increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients PMID: 22189597
  8. Mutation in 3-methylcrotonyl CoA carboxylase 1 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
  9. study reports eight different mutant alleles of MCCC1 or MCCC2 including six novel mutations in Korean patients with 3-methylcrotonyl-CoA carboxylase (MCC) deficiency PMID: 22150417
  10. identified two novel MCCA and four novel MCCB mutant alleles from five MCC-deficient patients PMID: 21071250
  11. factors other than the genotype at the MCCA and MCCB loci have a major influence on the phenotype of MCC deficiency PMID: 16010683
  12. The amino-termini containing 39 (MCCalpha) or 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
  13. Molecular analyses revealed novel mutations in one of the causative genes, MCCA or MCCB, in all five of the MCC deficiency patients PMID: 17968484
  14. A family with 3-methylcrotonyl-CoA carboxylase deficiency with different clinical features is described. PMID: 19339287
  15. analysis of a novel mechanism causing 3-methylcrotonyl-CoA carboxylase deficiency PMID: 19706617

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Involvement in disease
3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D)
Subcellular Location
Mitochondrion matrix.
Database Links

HGNC: 6936

OMIM: 210200

KEGG: hsa:56922

STRING: 9606.ENSP00000265594

UniGene: Hs.47649

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