[HTML][HTML] Mitochondrial ATP synthase: architecture, function and pathology
AI Jonckheere, JAM Smeitink… - Journal of inherited …, 2012 - Springer
Human mitochondrial (mt) ATP synthase, or complex V consists of two functional domains: F
1, situated in the mitochondrial matrix, and F o, located in the inner mitochondrial …
1, situated in the mitochondrial matrix, and F o, located in the inner mitochondrial …
Mitochondrial respiratory chain complexes
JS Sousa, E D'Imprima, J Vonck - Membrane protein complexes: structure …, 2018 - Springer
Mitochondria are the power stations of the eukaryotic cell, using the energy released by the
oxidation of glucose and other sugars to produce ATP. Electrons are transferred from NADH …
oxidation of glucose and other sugars to produce ATP. Electrons are transferred from NADH …
Selectivity of TMC207 towards mycobacterial ATP synthase compared with that towards the eukaryotic homologue
AC Haagsma, R Abdillahi-Ibrahim… - Antimicrobial agents …, 2009 - Am Soc Microbiol
The diarylquinoline TMC207 kills Mycobacterium tuberculosis by specifically inhibiting ATP
synthase. We show here that human mitochondrial ATP synthase (50% inhibitory …
synthase. We show here that human mitochondrial ATP synthase (50% inhibitory …
[HTML][HTML] Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects
E Fernández-Vizarra, V Tiranti, M Zeviani - Biochimica et Biophysica Acta …, 2009 - Elsevier
Assembly of the oxidative phosphorylation (OXPHOS) system in the mitochondrial inner
membrane is an intricate process in which many factors must interact. The OXPHOS system …
membrane is an intricate process in which many factors must interact. The OXPHOS system …
[HTML][HTML] Alleviation of neuronal energy deficiency by mTOR inhibition as a treatment for mitochondria-related neurodegeneration
mTOR inhibition is beneficial in neurodegenerative disease models and its effects are often
attributable to the modulation of autophagy and anti-apoptosis. Here, we report a neglected …
attributable to the modulation of autophagy and anti-apoptosis. Here, we report a neglected …
[HTML][HTML] ATP synthase diseases of mitochondrial genetic origin
Devastating human neuromuscular disorders have been associated to defects in the ATP
synthase. This enzyme is found in the inner mitochondrial membrane and catalyzes the last …
synthase. This enzyme is found in the inner mitochondrial membrane and catalyzes the last …
A cell biological perspective on mitochondrial dysfunction in Parkinson disease and other neurodegenerative diseases
W Mandemakers, VA Morais… - Journal of cell …, 2007 - journals.biologists.com
Dysfunction of mitochondria is frequently proposed to be involved in neurodegenerative
disease. Deficiencies in energy supply, free radical generation, Ca2+ buffering or control of …
disease. Deficiencies in energy supply, free radical generation, Ca2+ buffering or control of …
Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies
Mitochondrial diseases are severe and largely untreatable. Owing to the many essential
processes carried out by mitochondria and the complex cellular systems that support these …
processes carried out by mitochondria and the complex cellular systems that support these …
The mitochondrial respiratory chain is a modulator of apoptosis
JQ Kwong, MS Henning, AA Starkov… - The Journal of cell …, 2007 - rupress.org
Mitochondrial dysfunction and dysregulation of apoptosis are implicated in many diseases
such as cancer and neurodegeneration. We investigate here the role of respiratory chain …
such as cancer and neurodegeneration. We investigate here the role of respiratory chain …
[HTML][HTML] Mitochondrial ATP synthase disorders: molecular mechanisms and the quest for curative therapeutic approaches
R Kucharczyk, M Zick, M Bietenhader, M Rak… - … et Biophysica Acta (BBA …, 2009 - Elsevier
In mammals, the majority of cellular ATP is produced by the mitochondrial F1FO-ATP
synthase through an elaborate catalytic mechanism. While most subunits of this enzymatic …
synthase through an elaborate catalytic mechanism. While most subunits of this enzymatic …