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Heme (dys)homeostasis and liver disease.
Front Physiol
July 2024
i3S-Instituto de Investigação e Inovação em Saúde, Universidade do Porto, Porto, Portugal.
Heme is essential for a variety of proteins involved in vital physiological functions in the body, such as oxygen transport, drug metabolism, biosynthesis of steroids, signal transduction, antioxidant defense and mitochondrial respiration. However, free heme is potentially cytotoxic due to the capacity of heme iron to promote the oxidation of cellular molecules. The liver plays a central role in heme metabolism by significantly contributing to heme synthesis, heme detoxification, and recycling of heme iron.
View Article and Find Full Text PDFTeaching an old drug new tricks: Regulatory insights for the repurposing of hemin in cardiovascular disease.
Pharmacol Res Perspect
August 2024
Department of Pharmacology, Dalhousie University, Saint John, New Brunswick, Canada.
Drug repurposing has gained significant interest in recent years due to the high costs associated with de novo drug development; however, comprehensive pharmacological information is needed for the translation of pre-existing drugs across clinical applications. In the present study, we explore the current pharmacological understanding of the orphan drug, hemin, and identify remaining knowledge gaps with regard to hemin repurposing for the treatment of cardiovascular disease. Originally approved by the United States Food and Drug Administration in 1983 for the treatment of porphyria, hemin has attracted significant interest for therapeutic repurposing across a variety of pathophysiological conditions.
View Article and Find Full Text PDFDermatologic manifestations of hereditary hemochromatosis: A systematic review.
J Eur Acad Dermatol Venereol
May 2024
Faculty of Medicine, UNSW Medicine, University of New South Wales, Sydney, New South Wales, Australia.
Article Synopsis
- * The study reviewed 1582 publications from 1904 to 2023, narrowing it down to 22 studies, primarily case reports from the USA, UK, and France, involving 148 patients with skin symptoms associated with HH.
- * Key findings highlight diverse skin symptoms such as hyperpigmentation and resistant pruritus linked to iron deposition, while also indicating potential links between HH and skin cancers, underlining the importance of early identification for familial care.
Development and validation of diagnostic algorithms for the laboratory diagnosis of porphyrias.
J Inherit Metab Dis
November 2022
Clinical Department of Laboratory Medicine, University Hospitals Leuven, Leuven, Belgium.
Article Synopsis
- Porphyrias are uncommon metabolic disorders related to hemoglobin production, with symptoms that can be neurological, skin-related, or both, making diagnosis challenging.
- Researchers created and validated two diagnostic algorithms for acute and cutaneous porphyrias based on the symptoms presented, utilizing data from the UZ Leuven hospital and the European porphyria network.
- These algorithms demonstrated excellent sensitivity (100% for both types) and high specificity (98.5% for acute and 93.9% for cutaneous porphyrias) in accurately identifying cases, providing valuable support for clinicians.
A primer on heme biosynthesis.
Biol Chem
November 2022
Department of Biochemistry and Molecular Biology, University of Georgia, Athens, GA 30602-1111, USA.
Heme (protoheme IX) is an essential cofactor for a large variety of proteins whose functions vary from one electron reactions to binding gases. While not ubiquitous, heme is found in the great majority of known life forms. Unlike most cofactors that are acquired from dietary sources, the vast majority of organisms that utilize heme possess a complete pathway to synthesize the compound.
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