title: Heme content of normal and porphyric cultured skin fibroblasts.
abstract: Partial deficiencies in enzyme activities of the heme biosynthetic pathway have been demonstrated in cultured skin fibroblasts and other tissues from patients with protoporphyria (PP) and acute intermittent porphyria (AIP). We have quantitatively and qualitatively assessed the heme and free porphyrin content in cultured PP, AIP, VP (variegate porphyria, in which an enzymatic deficiency has not been identified), and normal skin fibroblasts during routine culture conditions in order to assess the overall metabolism of heme in these cells. The total heme concentration was not significantly different between control and porphyric lines; 189 +/- 15 pmoles/mg protein (mean +/- SEM) in controls, 154 +/- 17 in PP, 175 +/- 20 in AIP, and 181 +/- 81 in VP. The hemoprotein difference spectra were similar in all lines. Free porphyrins were not detected in any of the disorders. Despite partial deficiencies in enzyme activities of the heme pathway, porphyric fibroblasts thus maintain normal heme content during routine culture conditions without detectable porphyrin accumulation.
mesh_terms: D002478:Cells, Cultured; D003580:Cytochromes / Q000378:metabolism; D005347:Fibroblasts / Q000378:metabolism; D006418:Heme / Q000378:metabolism*; D006801:Humans; D011164:Porphyrias / Q000378:metabolism*; D011166:Porphyrins / Q000378:metabolism; D012867:Skin / Q000166:cytology / Q000378:metabolism*; D013057:Spectrum Analysis
chemical_list: D003580:Cytochromes; D011166:Porphyrins; D006418:Heme