Cob(I)alamin

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Cob(I)alamin
2D structure for Cob(I)alamin
Chemical Name
Chemical Formula	C₆₂H₈₈CoN₁₃O₁₄P
CAS Number	18534-66-2
Chemical Information	HMDB03429
Biochemical Taxonomy	Cobalamin Derivatives
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 0.0003 (0.00016-0.0005) umol/L
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
Cob(I)alamin is the substrate of the enzyme ATP:cob(I)alamin adenosyltransferase (EC 2.5.1.17), that converts reduced cob(I)alamin to the adenosylcobalamin co-factor required for the functional activity of methylmalonyl-CoA mutase (EC 5.4.99.2). Mutations in the human MMAB gene result in a block in adenosylcobalamin synthesis and are responsible for the cblB complementation group of inherited vitamin B12 disorders. Vitamin B12 (cobalamin) is a complex cobalt-containing molecule that is essential to human health. It is synthesized in bacteria where it catalyzes numerous methyl transfer and intramolecular rearrangement reactions. In mammals, it is the co-factor of only two enzymes: methionine synthase, which catalyzes the transfer of a methyl group from methyltetrahydrofolate to homocysteine to form methionine, and methylmalonyl-CoA mutase, which catalyzes the rearrangement of methylmalonyl-CoA to form succinyl-CoA. For both enzymes, the vitamin must be modified through intracellular metabolism to co-factor forms: methylcobalamin (MeCbl)1 for methionine synthase and adenosylcobalamin (AdoCbl) for methylmalonyl-CoA mutase. (PMID: 16439175)