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Shigeru Sassa, Recent progress in heme synthesis and metabolism, Stem Cells, Volume 12, Issue S1, 1994, Pages 1–10, https://doi.org/10.1002/stem.5530120704
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Abstract
Heme serves as the prosthetic group of various hemoproteins that carry out many essential functions for cells. For example, the transport of oxygen is carried out by hemoglobin and myoglobin; electron transport depends on the function of various mitochondrial cytochromes; oxidative metabolism in a number of xenobiotic substances and endogenous steroid hormones, vitamins and fatty acids are catalyzed by the activity of the microsomal cytochrome P450. In addition, heme is involved in the translation of proteins, and is required in certain aspects of cell development and differentiation. Inherited and acquired enzymatic defects in heme biosynthesis result in clinical conditions termed the porphyrias. Patients with porphyria express various difficulties in these functions as neurological disturbances, abnormal drug metabolism, and/or skin photosensitivity. Recent advances in this field have shed much light on the genetic, enzymological and clinical aspects of heme synthesis, catabolism and inherited defects of these enzymes.