OPA1: How much do we know to approach therapy?
V Del Dotto, M Fogazza, G Lenaers, M Rugolo… - Pharmacological …, 2018 - Elsevier
OPA1 is a GTPase that controls several functions, such as mitochondrial dynamics and
energetics, mtDNA maintenance and cristae integrity. In the last years, there have been …
energetics, mtDNA maintenance and cristae integrity. In the last years, there have been …
[HTML][HTML] Pharmacological inhibition of necroptosis protects from dopaminergic neuronal cell death in Parkinson's disease models
A Iannielli, S Bido, L Folladori, A Segnali, C Cancellieri… - Cell reports, 2018 - cell.com
Dysfunctions in mitochondrial dynamics and metabolism are common pathological
processes associated with Parkinson's disease (PD). It was recently shown that an inherited …
processes associated with Parkinson's disease (PD). It was recently shown that an inherited …
[HTML][HTML] The cristae modulator Optic atrophy 1 requires mitochondrial ATP synthase oligomers to safeguard mitochondrial function
R Quintana-Cabrera, C Quirin, C Glytsou… - Nature …, 2018 - nature.com
It is unclear how the mitochondrial fusion protein Optic atrophy 1 (OPA1), which inhibits
cristae remodeling, protects from mitochondrial dysfunction. Here we identify the …
cristae remodeling, protects from mitochondrial dysfunction. Here we identify the …
Mitochondrial disorders of the retinal ganglion cells and the optic nerve
J Finsterer, M Mancuso, D Pareyson, JM Burgunder… - Mitochondrion, 2018 - Elsevier
Objectives To summarise and discuss recent findings and future perspectives concerning
mitochondrial disorders (MIDs) affecting the retinal ganglion cells and the optic nerve …
mitochondrial disorders (MIDs) affecting the retinal ganglion cells and the optic nerve …
Short‐duration swimming exercise after myocardial infarction attenuates cardiac dysfunction and regulates mitochondrial quality control in aged mice
D Zhao, Y Sun, Y Tan, Z Zhang, Z Hou… - Oxidative medicine …, 2018 - Wiley Online Library
Background. Exercise benefits to cardiac rehabilitation (CR) following stable myocardial
infarction (MI). The suitable exercise duration for aged patients with coronary heart disease …
infarction (MI). The suitable exercise duration for aged patients with coronary heart disease …
[HTML][HTML] Mechanosensitivity of mitochondrial large-conductance calcium-activated potassium channels
A Walewska, B Kulawiak, A Szewczyk… - Biochimica et Biophysica …, 2018 - Elsevier
Potassium channels have been discovered in the inner mitochondrial membrane of various
cells. These channels can regulate the mitochondrial membrane potential, the matrix …
cells. These channels can regulate the mitochondrial membrane potential, the matrix …
[HTML][HTML] Deciphering OPA1 mutations pathogenicity by combined analysis of human, mouse and yeast cell models
V Del Dotto, M Fogazza, F Musiani, A Maresca… - … et Biophysica Acta (BBA …, 2018 - Elsevier
OPA1 is the major gene responsible for Dominant Optic Atrophy (DOA) and the syndromic
form DOA “plus”. Over 370 OPA1 mutations have been identified so far, although their …
form DOA “plus”. Over 370 OPA1 mutations have been identified so far, although their …
Retina-specific loss of Ikbkap/Elp1 causes mitochondrial dysfunction that leads to selective retinal ganglion cell degeneration in a mouse model of familial …
Y Ueki, V Shchepetkina… - Disease Models & …, 2018 - journals.biologists.com
Familial dysautonomia (FD) is an autosomal recessive disorder marked by developmental
and progressive neuropathies. It is caused by an intronic point-mutation in the IKBKAP/ELP1 …
and progressive neuropathies. It is caused by an intronic point-mutation in the IKBKAP/ELP1 …
Stem cell modeling of mitochondrial parkinsonism reveals key functions of OPA1
M Jonikas, M Madill, A Mathy, T Zekoll… - Annals of …, 2018 - Wiley Online Library
Objective Defective mitochondrial function attributed to optic atrophy 1 (OPA1) mutations
causes primarily optic atrophy and, less commonly, neurodegenerative syndromes. The …
causes primarily optic atrophy and, less commonly, neurodegenerative syndromes. The …
[HTML][HTML] ERK regulates mitochondrial membrane potential in fission deficient Drosophila follicle cells during differentiation
Mitochondrial morphology regulatory proteins interact with signaling pathways involved in
differentiation. In Drosophila oogenesis, EGFR signaling regulates mitochondrial …
differentiation. In Drosophila oogenesis, EGFR signaling regulates mitochondrial …