Western Blot: MCCC1 Antibody [NBP1-81254] - Lane 1: Marker [kDa] 230, 130, 95, 72, 56, 36, 28, 17, 11. Lane 2: Human cell line RT-4. Lane 3: Human cell line U-251MG sp
Immunocytochemistry/ Immunofluorescence: MCCC1 Antibody [NBP1-81254] - Immunofluorescent staining of human cell line U-2 OS shows localization to mitochondria. Antibody staining is shown in green.
Immunohistochemistry-Paraffin: MCCC1 Antibody [NBP1-81254] - Staining of human cerebral cortex shows strong cytoplasmic positivity in neuronal cells.
This antibody was developed against Recombinant Protein corresponding to amino acids: ITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFEAGNVHTDFIPQHH
Predicted Species
Rat (90%). Backed by our 100% Guarantee.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
MCCC1
Purity
Immunogen affinity purified
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Use in Mouse reported in scientific publication (PMID: 32561715).
Packaging, Storage & Formulations
Storage
Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
Buffer
PBS (pH 7.2) and 40% Glycerol
Preservative
0.02% Sodium Azide
Purity
Immunogen affinity purified
Alternate Names for MCCC1 Antibody
3-methylcrotonyl-CoA carboxylase 1
DKFZp686B20267
EC 6.4.1
EC 6.4.1.4
MCCAFLJ25545
MCC-B
methylcrotonoyl-CoA carboxylase 1 (alpha)
methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
mitochondrial
Background
MCCC1, also known as Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, is a 725 amino acid protein that is 80 kDa, located in mitochondrion matrix, functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Studies on this protein have shown a relationship with the following diseases and disorders: 3 methylcrotonyl-coa carboxylase 1 deficiency, pseudomyxoma peritonei, mucinous cystadenocarcinoma, cystadenocarcinoma, 3-methylcrotonyl-coa carboxylase deficiency, carnitine deficiency, neisseria meningitides, Parkinson's disease, hypotonia, pneumonia, and tuberculosis. This protein has also been shown to have interactions with PPP2R2B, YWHAB, ACADM, AUH and ECHS1 in valine, leucine and isoleucine degradation, metabolic pathways, metabolism of amino acids and derivatives, and branched-chain amino acid catabolism pathways.
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.
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