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Rabbit Anti-MCCC2 Antibody (CSB-PA867199ESR1HU)
Supplier: CUSABIO BIOTECH CO.
Methylcrotonoyl-CoA carboxylase beta chain Antibody,mitochondrial Antibody,MCCase subunit beta 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,MCCC2 Antibody,MCCB AntibodyMore alternative names for the 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|3-methylcrotonyl-CoA carboxylase 2 antibody|3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody|3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody|Biotin carboxylase antibody|MCCase subunit beta antibody|MCCB antibody|MCCB_HUMAN antibody|MCCC 2 antibody|Mccc2 antibody|Methylcrotonoyl CoA carboxylase 2 (beta) antibody|Methylcrotonoyl CoA carboxylase beta chain mitochondrial antibody|Methylcrotonoyl Coenzyme A carboxylase 2 (beta) antibody|Methylcrotonoyl-CoA carboxylase beta chain antibody|mitochondrial antibody|Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase antibody
Anti-MCCC2 antibody (ab197297)
Anti-MCCC2 antibody (ab197297)
Recommended applications: ELISA, WB, IHC
Recommended dilution: User optimized
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA)
ELISA, WB, IHC
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:64087( Human), Entrez Gene:78038( Mouse), Entrez Gene:361884( Rat), Omim:609014( Human), SwissProt:Q9HCC0( Human), SwissProt:Q3ULD5( Mouse), SwissProt:Q5XIT9( Rat), Unigene:604789( Human), Unigene:137327( Mouse), Unigene:33635( Rat)
|Protein 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. .
|Involvement in disease|| |
3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D) [MIM:210210]: An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the AccD/PCCB family.
|Protein cellular localization|| |
Mitochondrion matrix .
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CUSABIO BIOTECH CO.
|Product type|| |
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