aquocob(I)alamin vitamin B12s adenosyltransferase; ATP:cob(I)alamin adenosyltransferase; ATP:corrinoid adenosyltransferase; ATR; c-diamide adenosyltransferase; cblB; Cob; Cob(I)alamin adenosyltransferase; Cob(I)yrinic acid a; cob(I)yrinic acid a c diamide adenosyltransferase mitochondrial; Methylmalonic aciduria (cobalamin deficiency) cblB type; Methylmalonic aciduria type B protein; MGC20496, mitochondrial; MMAB; MMAB gene; MMAB_HUMAN; OTTHUMP00000240563; OTTHUMP00000240564

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

Polyclonal

IgG

Rabbit

MMAB Antibody detects endogenous levels of MMAB.

The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin (Thermo Fisher Scientific).

Liquid; Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

1mg/ml (lot specific)

Store at -20 degree C. Stable for 12 months from date of receipt.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Manufactured in a lab with traceable raw materials manufactured on site. Coordinated product portfolio of antibodies, pairs, conjugates, recombinant proteins, and immunoassay materials available, please inquire.

Small volumes of anti-MMAB 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.

Description: Adenosyltransferase involved in intracellular vitamin B12 metabolism. Generates adenosylcobalamin (AdoCbl) and directly delivers the cofactor to MUT in a transfer taht is stimulated by ATP-binding to MMAB and gated by MMAA.

Subunit Structure: Homotrimer.

Similarity: Belongs to the Cob(I)alamin adenosyltransferase family.

Western Blot (WB)

WB: 1:500-1:2000

Western blot analysis of extracts from Myeloma cells, using MMAB Antibody. The lane on the left was treated with blocking peptide.

Western blot analysis of extracts from HepG2 cells, using MMAB Antibody. The lane on the left was treated with blocking peptide.

Observed: 27 kDa
Predicted: 28 kDa

metabolism of cobalamin associated B

corrinoid adenosyltransferase

Cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial

MMAB_HUMAN

This gene encodes a protein that catalyzes the final step in the conversion of vitamin B(12) into adenosylcobalamin (AdoCbl), a vitamin B12-containing coenzyme for methylmalonyl-CoA mutase. Mutations in the gene are the cause of vitamin B12-dependent methylmalonic aciduria linked to the cblB complementation group. Alternatively spliced transcript variants have been found. [provided by RefSeq, Apr 2011]

MMAB: Defects in MMAB are the cause of methylmalonic aciduria type cblB (MMAB); also known as methylmalonic aciduria type B or vitamin B12-responsive methylmalonicaciduria of cblB complementation type. MMAB is a disorder of methylmalonate and cobalamin metabolism due to defective synthesis of adenosylcobalamin. Inheritance is autosomal recessive. Belongs to the Cob(I)alamin adenosyltransferase family.

Protein type: Motility/polarity/chemotaxis; EC 2.5.1.17; Transferase; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll

Chromosomal Location of Human Ortholog: 12q24

Cellular Component: mitochondrial matrix

Molecular Function: cob(I)yrinic acid a,c-diamide adenosyltransferase activity; ATP binding

Biological Process: vitamin metabolic process; cobalamin biosynthetic process; cobalamin metabolic process; water-soluble vitamin metabolic process

Disease: Methylmalonic Aciduria, Cblb Type

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