The enigma of the respiratory chain supercomplex

D Milenkovic, JN Blaza, NG Larsson, J Hirst - Cell metabolism, 2017 - cell.com
Respiratory chain dysfunction plays an important role in human disease and aging. It is now
well established that the individual respiratory complexes can be organized into …

[HTML][HTML] Structure and mechanism of mitochondrial electron transport chain

R Guo, J Gu, S Zong, M Wu, M Yang - Biomedical journal, 2018 - Elsevier
Respiration is one of the most vital and basic features of living organisms. In mammals,
respiration is accomplished by respiratory chain complexes located on the mitochondrial …

Accessory subunits are integral for assembly and function of human mitochondrial complex I

DA Stroud, EE Surgenor, LE Formosa, B Reljic… - Nature, 2016 - nature.com
Complex I (NADH: ubiquinone oxidoreductase) is the first enzyme of the mitochondrial
respiratory chain and is composed of 45 subunits in humans, making it one of the largest …

High-intensity training induces non-stoichiometric changes in the mitochondrial proteome of human skeletal muscle without reorganisation of respiratory chain content

C Granata, NJ Caruana, J Botella, NA Jamnick… - Nature …, 2021 - nature.com
Mitochondrial defects are implicated in multiple diseases and aging. Exercise training is an
accessible, inexpensive therapeutic intervention that can improve mitochondrial …

Mitochondrial respiratory complex I: structure, function and implication in human diseases

LK Sharma, J Lu, Y Bai - Current medicinal chemistry, 2009 - ingentaconnect.com
Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to
generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry …

Mitonuclear interactions: evolutionary consequences over multiple biological scales

JN Wolff, ED Ladoukakis… - … Transactions of the …, 2014 - royalsocietypublishing.org
Fundamental biological processes hinge on coordinated interactions between genes
spanning two obligate genomes—mitochondrial and nuclear. These interactions are key to …

The in-depth evaluation of suspected mitochondrial disease

TMMS Committee, RH Haas, S Parikh, MJ Falk… - Molecular genetics and …, 2008 - Elsevier
Mitochondrial disease confirmation and establishment of a specific molecular diagnosis
requires extensive clinical and laboratory evaluation. Dual genome origins of mitochondrial …

Mitochondrial phosphatase PTPMT1 is essential for cardiolipin biosynthesis

J Zhang, Z Guan, AN Murphy, SE Wiley, GA Perkins… - Cell metabolism, 2011 - cell.com
PTPMT1 was the first protein tyrosine phosphatase found localized to the mitochondria, but
its biological function was unknown. Herein, we demonstrate that whole body deletion of …

Analysis of the assembly profiles for mitochondrial-and nuclear-DNA-encoded subunits into complex I

M Lazarou, M McKenzie, A Ohtake… - … and cellular biology, 2007 - Am Soc Microbiol
Complex I of the respiratory chain is composed of at least 45 subunits that assemble
together at the mitochondrial inner membrane. Defects in human complex I result in energy …

Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome

NJ Lake, BD Webb, DA Stroud, TR Richman… - The American Journal of …, 2017 - cell.com
The synthesis of all 13 mitochondrial DNA (mtDNA)-encoded protein subunits of the human
oxidative phosphorylation (OXPHOS) system is carried out by mitochondrial ribosomes …