Clinical guide and update on porphyrias

U Stölzel, MO Doss, D Schuppan - Gastroenterology, 2019 - Elsevier
Physicians should be aware of porphyrias, which could be responsible for unexplained
gastrointestinal, neurologic, or skin disorders. Despite their relative rarity and complexity …

Coping with hypoxemia: Could erythropoietin (EPO) be an adjuvant treatment of COVID-19?

J Soliz, EM Schneider-Gasser, C Arias-Reyes… - Respiratory physiology …, 2020 - Elsevier
A very recent epidemiological study provides preliminary evidence that living in habitats
located at 2500 m above sea level (masl) might protect from the development of severe …

[HTML][HTML] Ferrochelatase: mapping the intersection of iron and porphyrin metabolism in the mitochondria

CD Obi, T Bhuiyan, HA Dailey… - Frontiers in Cell and …, 2022 - frontiersin.org
Porphyrin and iron are ubiquitous and essential for sustaining life in virtually all living
organisms. Unlike iron, which exists in many forms, porphyrin macrocycles are mostly …

RNAi therapy with givosiran significantly reduces attack rates in acute intermittent porphyria

E Sardh, P Harper - Journal of Internal Medicine, 2022 - Wiley Online Library
Acute hepatic porphyria (AHP) is a group of inherited metabolic disorders that affect hepatic
heme biosynthesis. They are associated with attacks of neurovisceral manifestations that …

Glutamine via α-ketoglutarate dehydrogenase provides succinyl-CoA for heme synthesis during erythropoiesis

JS Burch, JR Marcero, JA Maschek… - Blood, The Journal …, 2018 - ashpublications.org
During erythroid differentiation, the erythron must remodel its protein constituents so that the
mature red cell contains hemoglobin as the chief cytoplasmic protein component. For this,∼ …

[HTML][HTML] PPTC7 maintains mitochondrial protein content by suppressing receptor-mediated mitophagy

NM Niemi, LR Serrano, LK Muehlbauer… - Nature …, 2023 - nature.com
PPTC7 is a resident mitochondrial phosphatase essential for maintaining proper
mitochondrial content and function. Newborn mice lacking Pptc7 exhibit aberrant …

Post-translational modifications on mitochondrial metabolic enzymes in cancer

Y Peng, H Liu, J Liu, J Long - Free Radical Biology and Medicine, 2022 - Elsevier
Mitochondrion is the powerhouse of the cell. The research of nearly a century has expanded
our understanding of mitochondrion, far beyond the view that mitochondrion is an important …

[HTML][HTML] New avenues of heme synthesis regulation

AE Medlock, HA Dailey - International Journal of Molecular Sciences, 2022 - mdpi.com
During erythropoiesis, there is an enormous demand for the synthesis of the essential
cofactor of hemoglobin, heme. Heme is synthesized de novo via an eight enzyme-catalyzed …

[HTML][HTML] The ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation …

CM Rondelli, M Perfetto, A Danoff, H Bergonia… - Journal of Biological …, 2021 - ASBMB
Heme plays a critical role in catalyzing life-essential redox reactions in all cells, and its
synthesis must be tightly balanced with cellular requirements. Heme synthesis in eukaryotes …

[HTML][HTML] Regulation of heme synthesis by mitochondrial homeostasis proteins

YY Yien, M Perfetto - Frontiers in Cell and Developmental Biology, 2022 - frontiersin.org
Heme plays a central role in diverse, life-essential processes that range from ubiquitous,
housekeeping pathways such as respiration, to highly cell-specific ones such as oxygen …