[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 …

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 …

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 …

[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 …

[HTML][HTML] Alleviation of neuronal energy deficiency by mTOR inhibition as a treatment for mitochondria-related neurodegeneration

X Zheng, L Boyer, M Jin, Y Kim, W Fan, C Bardy… - elife, 2016 - elifesciences.org
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 …

[HTML][HTML] ATP synthase diseases of mitochondrial genetic origin

A Dautant, T Meier, A Hahn… - Frontiers in …, 2018 - frontiersin.org
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 …

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 …

Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies

JP Lasserre, A Dautant, RS Aiyar… - Disease models & …, 2015 - journals.biologists.com
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 …

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 …

[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 …