Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts
D Cotán, MD Cordero, J Garrido‐Maraver… - The FASEB …, 2011 - Wiley Online Library
Mitochondrial encephalomyopathy, lactic acidosis, and stroke‐like episodes (MELAS) is a
mitochondrial disease most usually caused by point mutations in tRNA genes encoded by …
mitochondrial disease most usually caused by point mutations in tRNA genes encoded by …
Mitochondrial mutations: newly discovered players in neuronal degeneration
NZ Lax, DM Turnbull, AK Reeve - The Neuroscientist, 2011 - journals.sagepub.com
Mutations in mitochondrial DNA cause a number of neurological diseases with defined
neuropathology; however, mutations in this genome have also been found to be important in …
neuropathology; however, mutations in this genome have also been found to be important in …
Homeostatic p62 levels and inclusion body formation in CHCHD2 knockout mice
S Sato, S Noda, S Torii, T Amo, A Ikeda… - Human molecular …, 2021 - academic.oup.com
Inactivation of constitutive autophagy results in the formation of cytoplasmic inclusions in
neurones, but the relationship between impaired autophagy and Lewy bodies (LBs) remains …
neurones, but the relationship between impaired autophagy and Lewy bodies (LBs) remains …
[HTML][HTML] Pathogenesis and treatment of mitochondrial myopathies: recent advances
S Di Mauro - Acta Myologica, 2010 - ncbi.nlm.nih.gov
In this brief review, I have highlighted recent advances in several areas of mitochondrial
medicine, including mtDNA-related diseases, mendelian mitochondrial …
medicine, including mtDNA-related diseases, mendelian mitochondrial …
The R941L mutation in MYH14 disrupts mitochondrial fission and associates with peripheral neuropathy
W Almutawa, C Smith, R Sabouny, RB Smit, T Zhao… - …, 2019 - thelancet.com
Background Peripheral neuropathies are often caused by disruption of genes responsible
for myelination or axonal transport. In particular, impairment in mitochondrial fission and …
for myelination or axonal transport. In particular, impairment in mitochondrial fission and …
Late onset motoneuron disorder caused by mitochondrial Hsp60 chaperone deficiency in mice
R Magnoni, J Palmfeldt, JH Christensen, M Sand… - Neurobiology of …, 2013 - Elsevier
Cells rely on efficient protein quality control systems (PQCs) to maintain proper activity of
mitochondrial proteins. As part of this system, the mitochondrial chaperone Hsp60 assists …
mitochondrial proteins. As part of this system, the mitochondrial chaperone Hsp60 assists …
Mitochondrial dysfunction in Parkinson's disease: focus on mitochondrial DNA
O Buneeva, V Fedchenko, A Kopylov, A Medvedev - Biomedicines, 2020 - mdpi.com
Mitochondria, the energy stations of the cell, are the only extranuclear organelles, containing
their own (mitochondrial) DNA (mtDNA) and the protein synthesizing machinery. The …
their own (mitochondrial) DNA (mtDNA) and the protein synthesizing machinery. The …
Defective mitochondrial dynamics is an early event in skeletal muscle of an amyotrophic lateral sclerosis mouse model
Mitochondria are dynamic organelles that constantly undergo fusion and fission to maintain
their normal functionality. Impairment of mitochondrial dynamics is implicated in various …
their normal functionality. Impairment of mitochondrial dynamics is implicated in various …
Insights on altered mitochondrial function and dynamics in the pathogenesis of neurodegeneration
J McInnes - Translational neurodegeneration, 2013 - Springer
In neurons, mitochondria are enriched to provide energy and calcium buffering required for
synaptic transmission. Additionally, mitochondria localize to the synapse, where they are …
synaptic transmission. Additionally, mitochondria localize to the synapse, where they are …
Progressive loss of striatal neurons causes motor dysfunction in MND2 mutant mice and is not prevented by Bcl-2
S Rathke-Hartlieb, U Schlomann, P Heimann… - Experimental …, 2002 - Elsevier
The mouse mutant “motoneuron disease 2”(MND2, mnd2 on Chr 6) was originally
characterized as a spinal muscular atrophy (SMA) because degenerating motoneurons …
characterized as a spinal muscular atrophy (SMA) because degenerating motoneurons …