Results 31 to 40 of about 253,295 (285)
Recurrent De Novo NAHR Reciprocal Duplications in the ATAD3 Gene Cluster Cause a Neurogenetic Trait with Perturbed Cholesterol and Mitochondrial Metabolism. [PDF]
, 2020 Recent studies have identified both recessive and dominant forms of mitochondrial disease that result from ATAD3A variants. The recessive form includes subjects with biallelic deletions mediated by non-allelic homologous recombination.
Armstrong, C +28 more
core +3 more sources
New insights into mitochondrial fusion [PDF]
FEBS Letters, 2007 Fusion controls mitochondrial morphology and is important for normal mitochondrial function, including roles in respiration, development, and apoptosis. Key components of the mitochondrial fusion machinery have been identified, allowing an initial dissection of its molecular mechanism. Outer and inner membrane fusion events are coordinately coupled but
Zhang, Yan, Chan, David C.
openaire +2 more sources
Proteolytic Processing of OPA1 Links Mitochondrial Dysfunction to Alterations in Mitochondrial Morphology [PDF]
, 2006 Many muscular and neurological disorders are associated with mitochondrial dysfunction and are often accompanied by changes in mitochondrial morphology.
Attardi, Giuseppe +11 more
core +2 more sources
Emerging functions of mammalian mitochondrial fusion and fission [PDF]
, 2005 Mitochondria provide a myriad of services to the cell, including energy production, calcium buffering and regulation of apoptosis. How these diverse functions are coordinated among the hundreds of mitochondria in a given cell is largely unknown, but is ...
Chan, David C., Chen, Hsiuchen
core +1 more source
Complementation between mouse Mfn1 and Mfn2 protects mitochondrial fusion defects caused by CMT2A disease mutations [PDF]
, 2007 Mfn2, an oligomeric mitochondrial protein important for mitochondrial fusion, is mutated in Charcot-Marie-Tooth disease (CMT) type 2A, a peripheral neuropathy characterized by axonal degeneration.
Chan, David C., Detmer, Scott A.
core +2 more sources
Regulation of Mitochondrial Dynamics and Neurodegenerative Diseases [PDF]
, 2011 Mitochondria are important cellular organelles in most metabolic processes and have a highly dynamic nature, undergoing frequent fission and fusion. The dynamic balance between fission and fusion plays critical roles in mitochondrial functions. In recent
Fujimura, Atsushi +6 more
core +1 more source
Frontiers in Cell and Developmental Biology, 2022 Mitochondrial repair is essential to metabolic homeostasis. Outer mitochondrial membrane mitofusin (MFN) proteins orchestrate mitochondrial fusion that opposes mitochondrial degeneration caused by senescence.
Jiajia Li +3 more
doaj +1 more source
Domain interactions within Fzo1 oligomers are essential for mitochondrial fusion [PDF]
, 2006 Mitofusins are conserved GTPases essential for the fusion of mitochondria. These mitochondrial outer membrane proteins contain a GTPase domain and two or three regions with hydrophobic heptad repeats, but little is known about how these domains interact ...
Chan, David C., Griffin, Erik E.
core +3 more sources
Neuronal hypoxia disrupts mitochondrial fusion
Neuroscience, 2015 Brain ischemia/reperfusion injury results in death of vulnerable neurons and extensive brain damage. It is well known that mitochondrial release of cytochrome c (cyto c) is a hallmark of neuronal death, however the molecular events underlying this release are largely unknown. We tested the hypothesis that cyto c release is regulated by breakdown of the
Sanderson, T.H. +2 more
openaire +2 more sources
Mitochondrial Dynamics in Mitochondrial Diseases
Diseases, 2016 Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial ...
Juan M. Suárez-Rivero +9 more
doaj +1 more source