Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

EC Genin, B Madji Hounoum, S Bannwarth… - Acta …, 2019 - Springer
… Recently, we provided genetic basis showing that mitochondrial dysfunction can trigger motor
neuron degeneration, through identification of CHCHD10 encoding a mitochondrial protein…

[HTML][HTML] Mitochondrial mechanisms of neuromuscular junction degeneration with aging

ME Anagnostou, RT Hepple - Cells, 2020 - mdpi.com
… in mitochondria within the motoneurons impact aging muscle is less evolved. Indeed, most
of our understanding in the context of mitochondrialjunction degeneration, mitochondrial

[HTML][HTML] Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons

L Dupuis, JL Gonzalez De Aguilar, A Echaniz-Laguna… - PloS one, 2009 - journals.plos.org
… To further explore the negative influence of muscle mitochondrial uncoupling on motor
neuron degeneration, we analysed whether muscle UCP1 overexpression could change the …

[HTML][HTML] Increased superoxide in vivo accelerates age-associated muscle atrophy through mitochondrial dysfunction and neuromuscular junction degeneration

YC Jang, MS Lustgarten, Y Liu, FL Muller… - The FASEB …, 2010 - ncbi.nlm.nih.gov
… Despite the increase in content, the function of mitochondria is … induced NMJ degeneration
and mitochondrial dysfunction are … -induced NMJ degeneration and mitochondrial dysfunction …

Mitochondrial DNA mutations in human degenerative diseases and aging

DC Wallace, JM Shoffner, I Trounce, MD Brown… - … et Biophysica Acta (BBA …, 1995 - Elsevier
… A wide variety of mitochondrial DNA (mtDNA) mutations have recently been identified in
degenerative diseases of the brain, … It has the same breakpoint junction as the 6.1 kb insertion. …

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice

E So, JC Mitchell, C Memmi, G Chennell… - Human molecular …, 2018 - academic.oup.com
… Whatever the mechanisms our results support the hypothesis that mitochondrial dysfunction
and early degeneration of the NMJ is a convergent event in the pathogenesis of ALS (26,28,…

Mitochondrial dynamics in neurodegeneration

K Itoh, K Nakamura, M Iijima, H Sesaki - Trends in cell biology, 2013 - cell.com
… Neurons have a high demand for mitochondrial metabolism and contain many mitochondria
throughout the cytoplasm. In neurons and many other cell types mitochondria are …

Disturbed mitochondrial dynamics and neurodegenerative disorders

F Burté, V Carelli, PF Chinnery… - Nature reviews …, 2015 - nature.com
… the narrow junctional regions that insulate the mitochondrial … the mitochondrial network due
to unopposed mitochondrial … leads to optic nerve degeneration and subsequent visual failure…

Impaired mitochondrial biogenesis, defective axonal transport of mitochondria, abnormal mitochondrial dynamics and synaptic degeneration in a mouse model of …

MJ Calkins, M Manczak, P Mao… - Human molecular …, 2011 - academic.oup.com
… synaptic mitochondria causes synaptic mitochondrial failure and synaptic degeneration in
… The purpose of this study was to better understand the effects of Aβ in mitochondrial activity …

Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants

JC Greene, AJ Whitworth, I Kuo… - Proceedings of the …, 2003 - National Acad Sciences
… from a spermatid individualization defect at a late stage of spermatogenesis. Mitochondrial
pathology is the earliest manifestation of muscle degeneration and a prominent characteristic …