Cob(I)yrinic Acid A,C-Diamide Adenosyltransferase, Mitochondrial (MMAB), ELISA

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

Store all reagents at 2-8 degree C

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Small volumes of MMAB elisa kit 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.

MBS9343633 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Cob(I)yrinic Acid A,C-Diamide Adenosyltransferase, Mitochondrial (MMAB) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MMAB. The ELISA analytical biochemical technique of the MBS9343633 kit is based on MMAB antibody-MMAB antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MMAB antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MMAB. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

27,388 Da

methylmalonic aciduria (cobalamin deficiency) cblB type

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

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

Chromosomal Location of Human Ortholog: 12q24

Cellular Component: mitochondrial matrix

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

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

Disease: Methylmalonic Aciduria, Cblb Type

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