In first step of haem synthesis, which amino acid is required ?
Synthesis of heme: In first step of Haem synthesis, Glycine amino acid is required Glycine condenses with succinyl CoA to form 6-amino levulinate which serves as a precursor for heme synthesis.. Copper is necessary for the synthesis of haemoglobin (Cu is a constituent of ALA synthase, needed for heme synthesis.) Pyridoxal phosphate is required for the synthesis of d-amino levulinic acid (the precursor for heme) Conversion of Succinyl CoA to a-Amino-b-ketoadipate requires pyridoxal phosphate. This is the first step of synthesis of heme that takes place in the mitochondria. a-Amino-b-ketoadipate is rapidly decarboxylated to d-aminolevulinate. Succinyl-CoA+glycine- a-amino-b-ketoadipate+CoA-SH-d-aminolevulinate+CO2
Histidine
Folate
Glycine
Fe
HAEM synth and porphyrias