Application
Western blot analysis of MCCA expression in A-431 whole cell lysate (A) and mouse liver tissue extract (B).Application
Western blot analysis of MCCA expression in non-transfected: (A) and mouse MCCA transfected: (B) 293T whole cell lysates.| Product Name | MCCA Antibody (H-290) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 436-725 mapping at the C-terminus of MCCA of human origin |
| Modification Notes | 3-methylcrotonyl-CoA:carboxylase (MCC) is an enzyme crucial in the breakdown of the branched chain amino acid leucine. Methylcrotonoyl-CoA carbox-ylase ? chain (MCCA), also designated 3-methylcrotonyl-CoA carboxylase 1, is located in the mitochondrial matrix. MCCA functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. MCCA has a Biotin cofactor. The gene encoding for the 725 amino acid MCCA protein maps to chromosome 3q26-q28 and consists of 19 exons. Defects in this gene are associated with 3-methylcrotonylglycinuria (MCGI), an autosomal recessive disorder characterized by muscular hypotonia and atrophy. Human MCC deficiency, also inherited recessively, is characterized by 3-methylcrotonyl-CoA accumulation. Symptoms may be highly variable, ranging from completely asymptomatic to metabolic acidosis and death in infancy. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of MCCA of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA also reactive with additional species, including equine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 436-725 mapping at the C-terminus of MCCA of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 436-725 mapping at the C-terminus of MCCA of human origin |
| Entrez Gene | 56922 |
|---|
Application
Western blot analysis of MCCA expression in A-431 whole cell lysate (A) and mouse liver tissue extract (B).
Application
Western blot analysis of MCCA expression in non-transfected: (A) and mouse MCCA transfected: (B) 293T whole cell lysates.| Application Notes | recommended for detection of MCCA of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA also reactive with additional species, including equine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
