Update on heme biosynthesis, tissue-specific regulation, heme transport, relation to iron metabolism and cellular energy

Liver International, September 2024

Heme is an essential molecule in the biochemistry of life.

Defects in the enzymes of the metabolic pathway for heme responsible for the porphyrias. Recent research shows how heme moves within the cell and throughout the bodies of mammals. 

Authors

Audrey Belot, Herve Puy, Iqbal Hamza, Herbert L Bonkovsky

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Case-based discussion of the acute hepatic porphyrias: Updates on pathogenesis, diagnosis and management

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Evidence-based consensus guidelines for the diagnosis and management of protoporphyria-related liver dysfunction in ePP & XLP