MCCA; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against MCCC1. It has been selected for its ability to recognize MCCC1 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4, Containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-MCCC1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-1:5000

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded stomach tissue)

79,344 Da

methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Mcca; MCCase subunit alpha??[Similar Products]

MCCC1: Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D). An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic *****s. 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.

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4; Ligase; Mitochondrial

Chromosomal Location of Human Ortholog: 3|3 B

Cellular Component: methylcrotonoyl-CoA carboxylase complex; mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: ATP binding; biotin carboxylase activity; catalytic activity; ligase activity; metal ion binding; methylcrotonoyl-CoA carboxylase activity; nucleotide binding

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