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Rabbit Anti-MCCC1 Antibody (CSB-PA853497ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Methylcrotonoyl-CoA carboxylase subunit alpha Antibody,mitochondrial Antibody,MCCase subunit alpha Antibody,3-methylcrotonyl-CoA carboxylase 1 Antibody,3-methylcrotonyl-CoA carboxylase biotin-containing subunit Antibody,3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha Antibody,MCCC1 Antibody,MCCA AntibodyMore alternative names for the antibody
3-methylcrotonyl-CoA carboxylase 1 antibody|3-methylcrotonyl-CoA carboxylase biotin-containing subunit antibody|3-methylcrotonyl-CoA carboxylase, alpha antibody|3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha antibody|DKFZp686B20267 antibody|FLJ25545 antibody|MCC-B antibody|MCCA antibody|MCCase subunit alpha antibody|methylcrotonoyl-CoA carboxylase 1 (alpha) antibody|methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial antibody|methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) antibody
Anti-MCCC1 antibody (ab178675)
Anti-MCCC1 antibody (ab178675)
Recommended applications: ELISA, WB, IHC
Recommended dilution: User optimized
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (526-725AA)
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:56922( Human), Entrez Gene:72039( Mouse), Entrez Gene:294972( Rat), Omim:609010( Human), SwissProt:Q96RQ3( Human), SwissProt:Q99MR8( Mouse), SwissProt:Q5I0C3( Rat), Unigene:47649( Human), Unigene:249016( Mouse), Unigene:15587( Rat)
|Protein function|| |
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. .
|Involvement in disease|| |
3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D) [MIM:210200]: 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 cellular localization|| |
Mitochondrion matrix .
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|Product type|| |
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