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) …
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …
Mitochondrial structure and bioenergetics in normal and disease conditions
M Protasoni, M Zeviani - International journal of molecular sciences, 2021 - mdpi.com
Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and
involved in various aspects of cellular life, with a primary role in energy production. The …
involved in various aspects of cellular life, with a primary role in energy production. The …
[HTML][HTML] Neural stem cells traffic functional mitochondria via extracellular vesicles
L Peruzzotti-Jametti, JD Bernstock, CM Willis… - PLoS …, 2021 - journals.plos.org
Neural stem cell (NSC) transplantation induces recovery in animal models of central
nervous system (CNS) diseases. Although the replacement of lost endogenous cells was …
nervous system (CNS) diseases. Although the replacement of lost endogenous cells was …
OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases
Patients with primary mitochondrial oxidative phosphorylation (OxPhos) defects present with
fatigue and multi-system disorders, are often lean, and die prematurely, but the mechanistic …
fatigue and multi-system disorders, are often lean, and die prematurely, but the mechanistic …
Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes
A Signes, E Fernandez-Vizarra - Essays in biochemistry, 2018 - portlandpress.com
The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
Mitochondrial diseases in man and mouse
DC Wallace - Science, 1999 - science.org
Over the past 10 years, mitochondrial defects have been implicated in a wide variety of
degenerative diseases, aging, and cancer. Studies on patients with these diseases have …
degenerative diseases, aging, and cancer. Studies on patients with these diseases have …
[图书][B] Muscle biopsy: a practical approach: expert consult; online and print
V Dubowitz, CA Sewry, A Oldfors - 2013 - books.google.com
2014 BMA Medical Book Awards Highly Commended in Pathology category! Muscle Biopsy:
A Practical Approach gives you all of the unparalleled guidance necessary to effectively …
A Practical Approach gives you all of the unparalleled guidance necessary to effectively …
Respiratory active mitochondrial supercomplexes
R Acín-Pérez, P Fernández-Silva, ML Peleato… - Molecular cell, 2008 - cell.com
The structural organization of the mitochondrial respiratory complexes as four big
independently moving entities connected by the mobile carriers CoQ and cytochrome c has …
independently moving entities connected by the mobile carriers CoQ and cytochrome c has …
Mitochondrial disease
AHV Schapira - The Lancet, 2006 - thelancet.com
Defects of mitochondrial metabolism cause a wide range of human diseases that include
examples from all medical subspecialties. This review updates the topic of mitochondrial …
examples from all medical subspecialties. This review updates the topic of mitochondrial …
The clinical maze of mitochondrial neurology
Mitochondrial diseases involve the respiratory chain, which is under the dual control of
nuclear and mitochondrial DNA (mtDNA). The complexity of mitochondrial genetics provides …
nuclear and mitochondrial DNA (mtDNA). The complexity of mitochondrial genetics provides …