EC Number   |
Natural Substrates  |
|---|
   1.4.4.2 | glycine + H-protein-lipoyllysine |
- |
| 1.4.4.2 | glycine + H-protein-lipoyllysine |
P-protein is the actual glycine-decarboxylating enzyme and uses pyridoxal 5'-phosphate as a cofactor. CO2 is released in the reaction and the residual aminomethyl group is bound to the oxidized lipoamide arm of H-protein |
| 1.4.4.2 | glycine + lipoylprotein |
- |
| 1.4.4.2 | glycine + lipoylprotein |
reaction is stimulated by lipoic acid which is a functional group of the H-protein |
| 1.4.4.2 | glycine + lipoylprotein |
glycine decarboxylation catalyzed by P-protein alone is extremely low |
| 1.4.4.2 | glycine + lipoylprotein |
lipoyl protein: H-protein, lipoamide can also act as acceptor |
| 1.4.4.2 | glycine + [glycine-cleavage complex H protein]-N6-lipoyl-L-lysine |
- |
| 1.4.4.2 | more |
nonketotic hyperglycinaemia is an autosomal recessive disorder of glycine metabolism caused by a deficiency in the mitochondrial glycine cleavage enzyme. The majority of cases are caused by mutations in the P-protein |
| 1.4.4.2 | more |
in transgenic potato plants with an antisense reduction in P-protein of GDC, the decrease in photorespiratory decarboxylation is compensated for by an increase in respiratory decarboxylation in the light |
