WikiLectures:Causes of pathological conformation of proteins

"It is formed by the condensation of glycine and succinite (succinyl-CoA) via the intermediate adipate (2-amino-3-oxo-1,6-hexanedioic acid), which is subsequently decarboxylated (removes CO 2 ) to form 5-aminolevulinic acid."The reaction is catalyzed by aminolevulate synthase (ALA-synthase, ALAS), located in the mitochondrial matrix. Pyridoxal-5-phosphate acts as a cofactor, forming a temporary Schiff base with glycine. Enzyme activity is sensitive to vitamínu B6 deficiency. The product of this reaction is the starting precoursor of heme.

Související články

 * Hemoglobin