Mitochondrial protein dysfunction in pathogenesis of neurological diseases

L Wang, Z Yang, X He, S Pu, C Yang, Q Wu… - Frontiers in Molecular …, 2022 - frontiersin.org
Mitochondria are essential organelles for neuronal function and cell survival. Besides the
well-known bioenergetics, additional mitochondrial roles in calcium signaling, lipid …

[HTML][HTML] Cristae junction as a fundamental switchboard for mitochondrial ion signaling and bioenergetics

B Gottschalk, CT Madreiter-Sokolowski, WF Graier - Cell Calcium, 2022 - Elsevier
OPA1 and MICU1 are both involved in the regulation of mitochondrial Ca 2+ uptake and the
stabilization of the cristae junction, which separates the inner mitochondrial membrane into …

MICU1 controls spatial membrane potential gradients and guides Ca2+ fluxes within mitochondrial substructures

B Gottschalk, Z Koshenov… - Communications …, 2022 - nature.com
Mitochondrial ultrastructure represents a pinnacle of form and function, with the inner
mitochondrial membrane (IMM) forming isolated pockets of cristae membrane (CM) …

Alterations of mitochondrial network by cigarette smoking and E-cigarette vaping

M Kanithi, S Junapudi, SI Shah, A Matta Reddy… - Cells, 2022 - mdpi.com
Toxins present in cigarette and e-cigarette smoke constitute a significant cause of illnesses
and are known to have fatal health impacts. Specific mechanisms by which toxins present in …

LIN28A enhances regenerative capacity of human somatic tissue stem cells via metabolic and mitochondrial reprogramming

K Pieknell, YA Sulistio, N Wulansari… - Cell Death & …, 2022 - nature.com
Developing methods to improve the regenerative capacity of somatic stem cells (SSCs) is a
major challenge in regenerative medicine. Here, we propose the forced expression of …

Predicting mitochondrial dynamic behavior in genetically defined neurodegenerative diseases

GW Dorn, X Dang - Cells, 2022 - mdpi.com
Mitochondrial dynamics encompass mitochondrial fusion, fission, and movement.
Mitochondrial fission and fusion are seemingly ubiquitous, whereas mitochondrial …

[HTML][HTML] Omega-3 fatty acids promote neuroprotection, decreased apoptosis and reduced glial cell activation in the retina of a mouse model of OPA1-related …

M Kalogerou, S Ioannou, P Kolovos… - Experimental Eye …, 2022 - Elsevier
The purpose of this study was to evaluate the neuroprotective effects of omega-3
polyunsaturated fatty acid (ω3-PUFA) supplementation in a mouse model of OPA1 …

Modelling autosomal dominant optic atrophy associated with OPA1 variants in iPSC-derived retinal ganglion cells

PE Sladen, K Jovanovic, R Guarascio… - Human Molecular …, 2022 - academic.oup.com
Autosomal dominant optic atrophy (DOA) is the most common inherited optic neuropathy,
characterized by the preferential loss of retinal ganglion cells (RGCs), resulting in optic …

Neuron-periphery mitochondrial stress communication in aging and diseases

J Li, J Cui, Y Tian - Life Medicine, 2022 - academic.oup.com
The nervous system is the central hub of the body, detecting environmental and internal
stimuli to regulate organismal metabolism via communications to the peripheral tissues …

Mutations at a split codon in the GTPase-encoding domain of OPA1 cause dominant optic atrophy through different molecular mechanisms

N Weisschuh, V Marino, K Schäferhoff… - Human Molecular …, 2022 - academic.oup.com
Exonic (ie coding) variants in genes associated with disease can exert pathogenic effects
both at the protein and mRNA level, either by altering the amino acid sequence or by …