[HTML][HTML] Mitochondrial optic neuropathies–disease mechanisms and therapeutic strategies

P Yu-Wai-Man, PG Griffiths, PF Chinnery - Progress in retinal and eye …, 2011 - Elsevier
Leber hereditary optic neuropathy (LHON) and autosomal-dominant optic atrophy (DOA) are
the two most common inherited optic neuropathies in the general population. Both disorders …

Apoptosis-inducing factor: structure, function, and redox regulation

IF Sevrioukova - Antioxidants & redox signaling, 2011 - liebertpub.com
Apoptosis-inducing factor (AIF) is a flavin adenine dinucleotide-containing, NADH-
dependent oxidoreductase residing in the mitochondrial intermembrane space whose …

Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy

C Giordano, M Montopoli, E Perli, M Orlandi, M Fantin… - Brain, 2011 - academic.oup.com
Leber's hereditary optic neuropathy, the most frequent mitochondrial disease due to
mitochondrial DNA point mutations in complex I, is characterized by the selective …

OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution

G Elachouri, S Vidoni, C Zanna, A Pattyn… - Genome …, 2011 - genome.cshlp.org
Eukaryotic cells harbor a small multiploid mitochondrial genome, organized in nucleoids
spread within the mitochondrial network. Maintenance and distribution of mitochondrial DNA …

Mitochondrial dysfunction in glaucoma and emerging bioenergetic therapies

S Lee, NJ Van Bergen, GY Kong… - Experimental eye …, 2011 - Elsevier
The similarities between glaucoma and mitochondrial optic neuropathies have driven a
growing interest in exploring mitochondrial function in glaucoma. The specific loss of retinal …

Ionizing radiation accelerates Drp1-dependent mitochondrial fission, which involves delayed mitochondrial reactive oxygen species production in normal human …

S Kobashigawa, K Suzuki, S Yamashita - Biochemical and biophysical …, 2011 - Elsevier
Ionizing radiation is known to increase intracellular level of reactive oxygen species (ROS)
through mitochondrial dysfunction. Although it has been as a basis of radiation-induced …

Mitochondrial dysfunction and pathophysiology of Charcot–Marie–Tooth disease involving GDAP1 mutations

J Cassereau, A Chevrollier, N Gueguen… - Experimental …, 2011 - Elsevier
Charcot–Marie–Tooth (CMT) disease represents a large group of clinically and genetically
heterogeneous disorders leading to inherited peripheral neuropathies affecting motor and …

A history of mitochondrial diseases

S DiMauro - Journal of inherited metabolic disease, 2011 - Springer
This articles reviews the development of mitochondrial medicine from the premolecular era
(1962–1988), when mitochondrial diseases were defined on the basis of clinical …

[HTML][HTML] Melanopsin-expressing retinal ganglion cells: implications for human diseases

C La Morgia, FN Ross-Cisneros, J Hannibal… - Vision research, 2011 - Elsevier
In the last decade, there was the seminal discovery of melanopsin-expressing retinal
ganglion cells (mRGCs) as a new class of photoreceptors that subserve the …

The complex interplay between mitochondrial dynamics and cardiac metabolism

V Parra, H Verdejo, A Del Campo, C Pennanen… - Journal of bioenergetics …, 2011 - Springer
Mitochondria are highly dynamic organelles, capable of undergoing constant fission and
fusion events, forming networks. These dynamic events allow the transmission of chemical …