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] Getting out what you put in: Copper in mitochondria and its impacts on human disease

PA Cobine, SA Moore, SC Leary - … Et Biophysica Acta (BBA)-Molecular Cell …, 2021 - Elsevier
Mitochondria accumulate copper in their matrix for the eventual maturation of the
cuproenzymes cytochrome c oxidase and superoxide dismutase. Transport into the matrix is …

Mitochondrial cytochrome c oxidase biogenesis: Recent developments

A Timón-Gómez, E Nývltová, LA Abriata, AJ Vila… - Seminars in cell & …, 2018 - Elsevier
Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen
consumption and is essential for aerobic energy generation in the form of ATP. Human COX …

The molecular and cellular basis of copper dysregulation and its relationship with human pathologies

MT Maung, A Carlson, M Olea‐Flores… - The FASEB …, 2021 - Wiley Online Library
Copper (Cu) is an essential micronutrient required for the activity of redox‐active enzymes
involved in critical metabolic reactions, signaling pathways, and biological functions …

[HTML][HTML] Mitochondrial protein synthesis adapts to influx of nuclear-encoded protein

R Richter-Dennerlein, S Oeljeklaus, I Lorenzi… - Cell, 2016 - cell.com
Mitochondrial ribosomes translate membrane integral core subunits of the oxidative
phosphorylation system encoded by mtDNA. These translation products associate with …

The subunit composition and function of mammalian cytochrome c oxidase

B Kadenbach, M Hüttemann - Mitochondrion, 2015 - Elsevier
Cytochrome c oxidase (COX) from mammals and birds is composed of 13 subunits. The
three catalytic subunits I-III are encoded by mitochondrial DNA, the ten nuclear-coded …

Exogenous melatonin maintains leaf quality of postharvest Chinese flowering cabbage by modulating respiratory metabolism and energy status

X Tan, Z Fan, Z Zeng, W Shan, J Kuang, W Lu… - Postharvest Biology and …, 2021 - Elsevier
The efficacy of melatonin on postharvest quality of Chinese flowering cabbage was
investigated in this study. Treatment of Chinese cabbage with melatonin significantly …

[HTML][HTML] Identification and immunological characterization of cuproptosis-related molecular clusters in Alzheimer's disease

Y Lai, C Lin, X Lin, L Wu, Y Zhao, F Lin - Frontiers in aging …, 2022 - frontiersin.org
Introduction Alzheimer's disease is the most common dementia with clinical and
pathological heterogeneity. Cuproptosis is a recently reported form of cell death, which …

[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 …

[HTML][HTML] 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 …