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 …

[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 …

[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 …

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 …

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 …

[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 …

[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 …

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 …

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 …

[HTML][HTML] ERK regulates mitochondrial membrane potential in fission deficient Drosophila follicle cells during differentiation

D Tomer, R Chippalkatti, K Mitra, R Rikhy - Developmental biology, 2018 - Elsevier
Mitochondrial morphology regulatory proteins interact with signaling pathways involved in
differentiation. In Drosophila oogenesis, EGFR signaling regulates mitochondrial …