However, only traces of cyanocobalamin occur naturally in the human body, which contains primarily hydroxocobalamin, adenosylcobalamin
, and methylcobalamin.
Methylmalonic acid serves as an indicator of cobalamin status because its conversion to succinate requires adenosylcobalamin
On a biochemical level, the Cbl-C defect prevents the conversion of vitamin B12 into its most important metabolically active forms-methylcobalamin and adenosylcobalamin
You also want the nutrients to be in a form that they occur in nature if they are available--for example, natural folate (not folic acid), natural mixed tocopherols for vitamin E (not just alpha-tocopherol), natural mixed carotenoids (not just beta-carotene), natural vitamin B12 (methylcobalamin or adenosylcobalamin
, not cyanocobalamin or hydroxycobalamin), and chelated minerals.
is required for conversion of methylmalonyl CoA to succinyl CoA and abnormality in this will lead to fatty acid accumulation (45).
coli, in the presence of ATP, to reduce the oxidation state of the cobalt atom in cobalamin and produce adenosylcobalamin
Dysfunction of this complementation group is known to produce deficits in both coenzyme forms of B12 needed by the cells, methylcobalamin and adenosylcobalamin
There maybe coexisting methylmalonic aciduria in patients with combined intracellular methylcobalamin and adenosylcobalamin
is required for the conversion of methylmalonyl coenzyme A (methylmalonic acid) to succinyl coenzyme A.
A functional deficiency of vitamin B12 is produced, resulting in lowering of methylcobalamin and adenosylcobalamin
12] to the active coenzyme adenosylcobalamin
(cblA, B, C, and D) are amenable to treatment with [B.
a biochemist at the University of Minnesota, involve attachment of a radioactive isotope to an analogue of Vitamin B12, DTPA- adenosylcobalamin