Therapeutic options in hereditary optic neuropathies

G Amore, M Romagnoli, M Carbonelli, P Barboni… - Drugs, 2021 - Springer
Options for the effective treatment of hereditary optic neuropathies have been a long time
coming. The successful launch of the antioxidant idebenone for Leber's Hereditary Optic …

The oligomycin axis of mitochondrial ATP synthase: OSCP and the proton channel

RJ Devenish, M Prescott, GM Boyle… - Journal of bioenergetics …, 2000 - Springer
Oligomycin has long been known as an inhibitor of mitochondrial ATP synthase, putatively
binding the F o subunits 9 and 6 that contribute to proton channel function of the complex. As …

[HTML][HTML] Insights into ATP synthase assembly and function through the molecular genetic manipulation of subunits of the yeast mitochondrial enzyme complex

RJ Devenish, M Prescott, X Roucou… - Biochimica et Biophysica …, 2000 - Elsevier
Development of an increasingly detailed understanding of the eucaryotic mitochondrial ATP
synthase requires a detailed knowledge of the stoichiometry, structure and function of F0 …

[HTML][HTML] Gene therapies for the treatment of Leber hereditary optic neuropathy

JA Sahel, NJ Newman, P Yu-Wai-Man… - International …, 2021 - journals.lww.com
J.-AS is supported by the Agence Nationale de la Recherche within the Programme
Investissements d'Avenir, Institut Hospitalo Universitaire FOReSIGHT (ANR-18-IAHU-0001) …

The constraints of allotopic expression

F Nieto-Panqueva, D Rubalcava-Gracia, PP Hamel… - Mitochondrion, 2023 - Elsevier
Allotopic expression is the functional transfer of an organellar gene to the nucleus, followed
by synthesis of the gene product in the cytosol and import into the appropriate organellar …

[HTML][HTML] Allotopic expression of mitochondrial genes: Basic strategy and progress

IM Artika - Genes & Diseases, 2020 - Elsevier
Allotopic expression of mitochondrial genes is a deliberate functional relocation of
mitochondrial genes into the nucleus followed by import of the gene-encoded polypeptide …

Current understanding of structure, function and biogenesis of yeast mitochondrial ATP synthase

IM Artika - Journal of bioenergetics and biomembranes, 2019 - Springer
The yeast mitochondrial ATP synthase is a rotary molecular machine primarily responsible
for the production of energy used to drive cellular processes. The enzyme complex is …

What limits the allotopic expression of nucleus-encoded mitochondrial genes? The case of the chimeric Cox3 and Atp6 genes

F Figueroa-Martínez, M Vázquez-Acevedo… - Mitochondrion, 2011 - Elsevier
Allotopic expression is potentially a gene therapy for mtDNA-related diseases. Some
OXPHOS proteins like ATP6 (subunit a of complex V) and COX3 (subunit III of complex IV) …

Cell and animal models of mtDNA biology: progress and prospects

SM Khan, RM Smigrodzki… - American Journal of …, 2007 - journals.physiology.org
The past two decades have witnessed an evolving understanding of the mitochondrial
genome's (mtDNA) role in basic biology and disease. From the recognition that mutations in …

[HTML][HTML] A cytochrome c–GFP fusion is not released from mitochondria into the cytoplasm upon expression of Bax in yeast cells

X Roucou, M Prescott, RJ Devenish, P Nagley - FEBS letters, 2000 - Elsevier
To study Bax-induced release of cytochrome c in vivo, we have expressed a cytochrome c–
GFP (green fluorescent protein) fusion in Saccharomyces cerevisiae cells null for the …