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Correlation with reticulocyte percentage was poor, in the aggregate a general trend toward increased values of both isomers I and III was noted with increasing reticulocytes. When the percent of type III was low the net amount was increased as compared with normal. Hemolysates of non\u2010porphyric, reticulocyte\u2010rich red cells (hemolytic or posthemorrhagic anemia) formed only minute amounts of type I porphyrin but at the same time no more, or even less type III than the porphyric hemolysates, although representing red cells of greater reticulocyte content. No evidence of deficient heme synthesis was observed in porphyric hemolysates incubated with [<jats:sup>14<\/jats:sup> C] \u2010porphobilinogen or <jats:sup>59<\/jats:sup>Fe. Other studies of porphyric hemolysates incubated with and without added mouse spleen synthetase failed to reveal evidence of an absolute UPG\u2010III cosynthetase (Co\u2010S) deficiency. The large increases of type I porphyrin with normal or increased formation of type III, both in the disease and in the hemolysates, are believed due to a primary increase of ALA\u2010S or UPG\u2010S activity rather than a decrease of Co\u2010S. Possible mutations which might be responsible for this increase are considered.<\/jats:p>","DOI":"10.1002\/ajh.2830010103","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T04:53:23Z","timestamp":1172033603000},"page":"3-21","source":"Crossref","is-referenced-by-count":24,"title":["The activities of uroporphyrinogen synthetase and cosynthetase in congenital erythropoietic porphyria (cep)"],"prefix":"10.1002","volume":"1","author":[{"given":"Ken","family":"Miyagi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Z. 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