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 …

[PDF][PDF] The protein import machinery of mitochondria—a regulatory hub in metabolism, stress, and disease

AB Harbauer, RP Zahedi, A Sickmann, N Pfanner… - Cell metabolism, 2014 - cell.com
Mitochondria fulfill central functions in bioenergetics, metabolism, and apoptosis. They
import more than 1,000 different proteins from the cytosol. It had been assumed that the …

[PDF][PDF] Mitochondrial and nuclear accumulation of the transcription factor ATFS-1 promotes OXPHOS recovery during the UPRmt

AM Nargund, CJ Fiorese, MW Pellegrino, P Deng… - Molecular cell, 2015 - cell.com
Mitochondrial diseases and aging are associated with defects in the oxidative
phosphorylation machinery (OXPHOS), which are the only complexes composed of proteins …

Mitochondria—Striking a balance between host and endosymbiont

RJ Youle - Science, 2019 - science.org
BACKGROUND Evolution generally trundles along by selecting for mutations or by
leveraging the new realms generated from chromosomal amplifications and the transfer of …

Organization and regulation of mitochondrial protein synthesis

M Ott, A Amunts, A Brown - Annual review of biochemistry, 2016 - annualreviews.org
Mitochondria are essential organelles of endosymbiotic origin that are responsible for
oxidative phosphorylation within eukaryotic cells. Independent evolution between species …

Nuclear miRNA regulates the mitochondrial genome in the heart

S Das, M Ferlito, OA Kent, K Fox-Talbot… - Circulation …, 2012 - Am Heart Assoc
Rationale: Mitochondria are semiautonomous cellular organelles with their own genome,
which not only supply energy but also participate in cell death pathways. MicroRNAs …

[PDF][PDF] 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 …

Protective coupling of mitochondrial function and protein synthesis via the eIF2α kinase GCN-2

BM Baker, AM Nargund, T Sun, CM Haynes - PLoS genetics, 2012 - journals.plos.org
Cells respond to defects in mitochondrial function by activating signaling pathways that
restore homeostasis. The mitochondrial peptide exporter HAF-1 and the bZip transcription …

Mitochondrial genetics

PF Chinnery, G Hudson - British medical bulletin, 2013 - academic.oup.com
Introduction In the last 10 years the field of mitochondrial genetics has widened, shifting the
focus from rare sporadic, metabolic disease to the effects of mitochondrial DNA (mtDNA) …

OXPHOS mutations and neurodegeneration

WJH Koopman, F Distelmaier, JAM Smeitink… - The EMBO …, 2013 - embopress.org
Mitochondrial oxidative phosphorylation (OXPHOS) sustains organelle function and plays a
central role in cellular energy metabolism. The OXPHOS system consists of 5 multisubunit …