1. Porphyrin biosynthesis and the enzymes succinyl-CoA synthetase (EC 6.2.1 S), Gaminolaevulinate synthase (EC 2.3.1.37), Gaminolaevulinate dehydratase (EC 4.2.1.24) and porphobilinogenase enzyme complex at 37°C and 45OC were studied in blood, bone marrow, spleen, liver and kidney of normal bulls and a bull with congenital erythropoietic porphyria (CEP). rinogen in the urine. The blood plasma contained only traces of porphyrin.
Enzyme determinations showed threefoldincreased succinyl-CoA synthase in CEP bone marrow as compared with normal animals, manyfold increase of aminolaevulinate synthase, normal aminolaevulinate dehydratase, sevenfold increase of porphobilinogenase activity on incubation at 45OC and threefold increase at 37OC. In blood, aminolaevulinate synthase and aminolaevulinate dehydratase were increased in CEP, whereas porphobilinogenase at 45OC was reduced. In liver, aminolaevulinate synthase was normal and porphobilinogenase decreased.
6.Our results show that in this animal with CEP there was an increased porphobilinogen deaminase and a significant enhancement of aminolaevulinate synthase concomitant with a diminished activity of uroporphyrinogen I11 cosynthase.