Protoporphyrin IX: the Good, the Bad, and the Ugly.
Galveston, United States. In J Pharmacol Exp Ther, Feb 2016
PPIX serves as the substrate for ferrochelatase, the final enzyme in heme biosynthesis, and its homeostasis is tightly regulated during heme synthesis.
[Heme metabolism as an integral part of iron homeostasis].
Panama. In Postepy Hig Med Dosw (online), 2013
The biogenesis of [Fe-S] centers is crucial for heme synthesis because these co-factors determine the activity of IRP1 and that of ferrochelatase, an enzyme responsible for the insertion of an iron into protoporphyrin IX to produce heme.
Erythropoietic Protoporphyria, Autosomal Recessive
Seattle, United States. In Unknown Journal, 2012
CLINICAL CHARACTERISTICS: Erythropoietic protoporphyria (EPP) is characterized by cutaneous photosensitivity (usually beginning in infancy or childhood) that results in tingling, burning, pain, and itching within minutes of sun/light exposure and may be accompanied by swelling and redness.
[Inheritance in erythropoietic protoporphyria].
Colombes, France. In Pathol Biol (paris), 2010
More than 96% of unrelated EPP patients have ferrochelatase deficiency (MIM 177000). Inheritance of a common hypomorphic IVS3-48C FECH allele trans to a deleterious FECH mutation reduces FECH activity below a critical threshold. Review.