Mitochondrial disorders of the OXPHOS system

E Fernandez‐Vizarra, M Zeviani - FEBS letters, 2021 - Wiley Online Library
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

[HTML][HTML] Structure, function, and assembly of heme centers in mitochondrial respiratory complexes

HJ Kim, O Khalimonchuk, PM Smith… - Biochimica et Biophysica …, 2012 - Elsevier
The sequential flow of electrons in the respiratory chain, from a low reduction potential
substrate to O2, is mediated by protein-bound redox cofactors. In mitochondria, hemes …

From synthesis to utilization: The ins and outs of mitochondrial heme

SA Swenson, CM Moore, JR Marcero, AE Medlock… - Cells, 2020 - mdpi.com
Heme is a ubiquitous and essential iron containing metallo-organic cofactor required for
virtually all aerobic life. Heme synthesis is initiated and completed in mitochondria, followed …

Mitochondrial protein synthesis, import, and assembly

TD Fox - Genetics, 2012 - academic.oup.com
The mitochondrion is arguably the most complex organelle in the budding yeast cell
cytoplasm. It is essential for viability as well as respiratory growth. Its innermost aqueous …

Cytochrome c Biogenesis: Mechanisms for Covalent Modifications and Trafficking of Heme and for Heme-Iron Redox Control

RG Kranz, C Richard-Fogal, JS Taylor… - … and molecular biology …, 2009 - Am Soc Microbiol
Heme is the prosthetic group for cytochromes, which are directly involved in
oxidation/reduction reactions inside and outside the cell. Many cytochromes contain heme …

Down the iron path: mitochondrial iron homeostasis and beyond

JV Dietz, JL Fox, O Khalimonchuk - Cells, 2021 - mdpi.com
Cellular iron homeostasis and mitochondrial iron homeostasis are interdependent.
Mitochondria must import iron to form iron–sulfur clusters and heme, and to incorporate …

Compensatory growth of healthy cardiac cells in the presence of diseased cells restores tissue homeostasis during heart development

JD Drenckhahn, QP Schwarz, S Gray, A Laskowski… - Developmental cell, 2008 - cell.com
Energy generation by mitochondrial respiration is an absolute requirement for cardiac
function. Here, we used a heart-specific conditional knockout approach to inactivate the X …

Metabolic pathways in the apicoplast of apicomplexa

F Seeber, D Soldati-Favre - International review of cell and molecular …, 2010 - Elsevier
Intracellular parasites of the phylum Apicomplexa harbor a plastid-like organelle called
apicoplast that is the most reduced organelle of this type known. Due to the medical …

Assembly factors of human mitochondrial respiratory chain complexes: physiology and pathophysiology

D Ghezzi, M Zeviani - … : Nuclear-Encoded Genes, Enzyme Regulation, and …, 2012 - Springer
Mitochondrial disorders are clinical syndromes associated with abnormalities of the
oxidative phosphorylation (OXPHOS) system, the main responsible for the production of …

[HTML][HTML] Reprint of: Biogenesis of the cytochrome bc1 complex and role of assembly factors

PM Smith, JL Fox, DR Winge - Biochimica et Biophysica Acta (BBA) …, 2012 - Elsevier
The cytochrome bc1 complex is an essential component of the electron transport chain in
most prokaryotes and in eukaryotic mitochondria. The catalytic subunits of the complex that …