Methylmalonic aciduria (cobalamin deficiency) cblB type; ATR; ATP:cob (I)alamin adenosyltransferase; Methylmalonic aciduria (cobalamin deficiency) cblB type

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

E Coli

> 95% by SDS-PAGE

Liquid. In 20 mM Tris-HCl Buffer (pH 7.5) containing 10% glycerol

1 mg/ml (determined by Bradford assay) (lot specific)

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

Small volumes of MMAB recombinant protein 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.

MMAB is 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 (MCM). Impaired MMAB activity leads to the inherited disorder vitamin B12 dependent methylmalonic aciduria linked to the cblB complementation group. Recombinant human MMAB protein, fused to His-tag at N-terminus, was expressed in E Coli and purified by using conventional chromatography.

Enzymes & Proteases

SDS-PAGE

26.3 kDa (239aa), confirmed by MALDI-TOF

methylmalonic aciduria (cobalamin deficiency) cblB type

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

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

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]

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.

Gravel RA., et al. (2009) Mol Genet Metab. 98 (3):278-84; Edwards AM., et al (2004) J Biol Chem. 279 (22):23646-53.;

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