To create this novel hydrogel, the team assembled genetically-engineered proteins into molecular networks by stitching together the photoreceptor C-terminal adenosylcobalamin
binding domain (CarHC) proteins at room temperature.
Cobalamin C (cblC) disease is the most common type of MMA and is characteristically concurrent with homocystinemia (HCY) due to impaired synthesis of two active forms of cbl, namely adenosylcobalamin
(AdoCbl) and methylcobalamin (MeCbl).
2] Nonstandard abbreviations: MS/MS, tandem mass spectrometry; MMA, methylmalonic acidemia; MMCoA, methylmalonyl-CoA; AdoCbl, 5-deoxy adenosylcobalamin
Supplement with: 500 mcg of vitamin B12* (look for methylcobalamin and adenosylcobalamin
, the most active forms).
There are numbers of naturally occurring Cbl species in the human body, but only methylcobalamin (MeCbl) and adenosylcobalamin
(AdoCbl) are biologically active, meaning that only they directly act as enzyme cofactors.
Cobalamin is used to denote the structure of a corrin nucleus; methylcobalamin and adenosylcobalamin
act as cofactors in the body, while the others are therapeutic methods of food consumption.
However, only traces of cyanocobalamin occur naturally in the human body, which contains primarily hydroxocobalamin, adenosylcobalamin
, and methylcobalamin.
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
The measurement of elevated levels of unmetabolized substrates of two enzymes dependent on the cofactors methylcobalamin (Figure 1) and adenosylcobalamin
(Figure 2) can be used to detect cobalamin deficiency.
GSCbl was significantly decreased at 1 hr and cyanocobalamin (CNCbl), adenosylcobalamin
(AdoCbl), and total Cbl were significantly decreased at 24 hrs.