Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing mitochondria localization.Application
Western blot analysis of MMAB expression in DU 145 (A) and Hep G2 (B) whole cell lysates and mouse skeletal muscle tissue extract (C).| Product Name | MMAB Antibody (H-217) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 34-250 mapping at the C-terminus of MMAB of human origin |
| Modification Notes | MMAB (methylmalonic aciduria (cobalamin deficiency) type B protein), also known as ATR or Cob(I)alamin adenosyltransferase, is a mitochondrial protein expressed in skeletal muscle and liver. MMAB belongs to the Cob(I)alamin adenosyltransferase family and plays an important role in adenosylcobalamin (AdoCbl) biosynthesis. More specifically, MMAB catalyzes the final step in the biosynthesis pathway: the conversion of vitamin B12 (also known as cobalamin) to AdoCbl. AdoCbl is an essential cofactor utilized by MUT, the mitochondrial methylmalonyl-CoA mutase that plays an important role in the catabolism of cholesterol, branched chain amino acids, odd-numbered fatty acids and other metabolites. Mutations in the gene encoding MMAB can result in methylmalonic aciduria type B (MMAB), also known as vitamin B12-responsive methylmalonicaciduria of cblB complementation type. The autosomal recessive MMAB disease is characterized by defective synthesis of AdoCbl. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of MMAB of mouse, rat and human origin by WB, IP, IF and ELISA: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 34-250 mapping at the C-terminus of MMAB of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 34-250 mapping at the C-terminus of MMAB of human origin |
| Entrez Gene | 326625 |
|---|
Application
Immunofluorescence staining of formalin-fixed Hep G2 cells showing mitochondria localization.
Application
Western blot analysis of MMAB expression in DU 145 (A) and Hep G2 (B) whole cell lysates and mouse skeletal muscle tissue extract (C).| Application Notes | recommended for detection of MMAB of mouse, rat and human origin by WB, IP, IF and ELISA: |
|---|
| 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. |
