Iron metabolism in the disorders of heme biosynthesis

A Ricci, G Di Betto, E Bergamini, E Buzzetti, E Corradini… - Metabolites, 2022 - mdpi.com
Given its remarkable property to easily switch between different oxidative states, iron is
essential in countless cellular functions which involve redox reactions. At the same time …

Iron, heme synthesis and erythropoietic porphyrias: a complex interplay

A Poli, C Schmitt, B Moulouel, A Mirmiran, H Puy… - Metabolites, 2021 - mdpi.com
Erythropoietic porphyrias are caused by enzymatic dysfunctions in the heme biosynthetic
pathway, resulting in porphyrins accumulation in red blood cells. The porphyrins deposition …

[HTML][HTML] Biology of heme in mammalian erythroid cells and related disorders

T Fujiwara, H Harigae - BioMed research international, 2015 - hindawi.com
Heme is a prosthetic group comprising ferrous iron (Fe 2+) and protoporphyrin IX and is an
essential cofactor in various biological processes such as oxygen transport (hemoglobin) …

Erythroid heme biosynthesis and its disorders

HA Dailey, PN Meissner - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
Heme, which is composed of iron and the small organic molecule protoporphyrin, is an
essential component of hemoglobin as well as a variety of physiologically important …

Recent progress in heme synthesis and metabolism

S Sassa - Stem Cells, 1994 - Wiley Online Library
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 …

Heme as key regulator of major mammalian cellular functions: molecular, cellular, and pharmacological aspects

AS Tsiftsoglou, AI Tsamadou… - Pharmacology & …, 2006 - Elsevier
Heme (iron protoporphyrin IX) exists as prosthetic group in several hemoproteins, which
include respiration cytochromes, gas sensors, P450 enzymes (CYPs), catalases …

Clinically important features of porphyrin and heme metabolism and the porphyrias

S Besur, W Hou, P Schmeltzer, HL Bonkovsky - Metabolites, 2014 - mdpi.com
Heme, like chlorophyll, is a primordial molecule and is one of the fundamental pigments of
life. Disorders of normal heme synthesis may cause human diseases, including certain …

Iron in porphyrias: friend or foe?

E Buzzetti, P Ventura, E Corradini - Diagnostics, 2022 - mdpi.com
Iron is a trace element that is important for many vital processes, including oxygen transport,
oxidative metabolism, cellular proliferation, and catalytic reactions. Iron supports these …

[PDF][PDF] Heme biosynthesis and its disorders: porphyrias and sideroblastic anemias

JS Wiley, MR Moore - Hoffman: Hematology: Basic Principles …, 2000 - ndl.ethernet.edu.et
The porphyrias and the sideroblastic anemias are metabolic disorders that both involve
defects in heme biosynthesis. Most forms of porphyria are inherited as Mendelian autosomal …

[HTML][HTML] Biochemistry, heme synthesis

AS Ogun, NV Joy, M Valentine - 2019 - europepmc.org
Heme is a porphyrin ring complexed with ferrous iron and protoporphyrin IX. Heme is an
essential prosthetic group in proteins that is necessary as a subcellular compartment to …