EC Number   |
Natural Substrates |
|---|
   1.14.18.2 | CMP-N-acetylneuraminate + 2 ferrocytochrome b5 + O2 + 2 H+ |
- |
| 1.14.18.2 | CMP-N-acetylneuraminate + ferrocytochrome b5 + O2 + H+ |
- |
| 1.14.18.2 | CMP-N-acetylneuraminate + ferrocytochrome b5 + O2 + H+ |
the enzyme determines the sugar bound to the red cell by converting NeuAc to NeuGc. Thus, mutations in CMAH may cause the A and B blood types, two major serotypes. Blood type A is dominant to type B. A rare type AB may also be allelic and is suspected to be recessive to A and dominant to B. Cat blood type antigens are defined: N-glycolylneuraminic acid, NeuGc, is associated with type A and N-acetylneuraminic acid, NeuAc, with type B, overview |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
- |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
binding of CMP-N-acetylneuraminate to CMP-N-acetylneuraminate hydroxylase changes conformation of the enzyme so as to construct a recognition site for cytochrome b5, followed by the formation of a ternary complex through this domain. Then the transport of electrons from NAD(P)H to the enzyme through cytochrome b5 takes place, CMP-N-acetylneuraminate is converted to CMP-N-glycoloylneuraminic acid and finally the ternary complex dissociates into its components to release CMP-N-glycoloylneuraminic acid |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
the enzyme is the key for regulation of the overall velocity of CMP-NeuAc hydroxylation and consequently for the expression of N-glycoloylneuraminic acid glycoconjugates |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
a regulation of CMP-N-acetylneuraminate hydroxylation and thus the ratio of glycoconjugate-bound N-acetylneuraminate and N-acetylglycoloylneuraminate might occur by varying the amount of hydroxylase protein within the cell, possibly by controlling the expression of the hydroxylase gene |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
the enzyme plays a decisive role in governing the relative amounts of N-acetylneuraminate and N-acetylglycolylneuraminate occuring in the glycoconjugates of a tissue |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
the biosynthesis of the sialic acid N-glycolylneuraminic acid occurs by the action of cytidine monophosphate-N-acetylneuraminate hydroxylase. Incorporation of N-glycoloylneuraminic acid into glycoconjugates is generally controlled by the amount of hydroxylase protein expressed in a tissue |
| 1.14.18.2 | CMP-N-acetylneuraminate + NADH + O2 |
key enzyme for the expression of N-glycoloylneuraminic acid |
