Results 71 to 80 of about 1,199,861 (319)

Fear of disease progression in carriers of the m.3243A > G mutation

Orphanet Journal of Rare Diseases, 2018
Background Being diagnosed with mitochondrial disease due to the m.3243A > G mutation is frequently preceded by a long diagnostic process. The disease itself is characterized by heterogeneous course and expression, so leaving patients with considerable ...
José A. E. Custers, Paul de Laat, Saskia Koene, Jan Smeitink, Mirian C. H. Janssen, Christianne Verhaak   +5 more
doaj   +1 more source

PARK(ing) time–How park deficiency affects the biological clock in a Drosophila model of Parkinson's disease

FEBS Letters, EarlyView.
Drosophila park mutants serve as a model for Parkinson's disease. We used this strain to investigate the connection between oxidative stress and the circadian clock mechanism. We showed that increased oxidative stress affects the physiology of pacemaker cells, disrupting their daily structural plasticity. Lack of rhythmic signaling from pacemaker cells
Kamila Zientara, Kinga Skoczek, Elzbieta Pyza, Milena Damulewicz   +3 more
wiley   +1 more source

Mitochondrial dynamics and mitochondrial quality control

Redox Biology, 2015
Mitochondria are cellular energy powerhouses that play important roles in maintaining cell survival, cell death and cellular metabolic homeostasis. Timely removal of damaged mitochondria via autophagy (mitophagy) is thus critical for cellular homeostasis and function.
Hong-Min Ni, Jessica A. Williams, Wen-Xing Ding   +2 more
openaire   +3 more sources

Mitochondrial Calcification

Immunometabolism, 2021
Abstract One of the most fascinating aspects of mitochondria is their remarkable ability to accumulate and store large amounts of calcium in the presence of phosphate leading to mitochondrial calcification. In this paper, we briefly address the mechanisms that regulate mitochondrial calcium homeostasis followed by the extensive
Duvvuri, Bhargavi, Lood, Christian
openaire   +2 more sources

TOM40 Mediates Mitochondrial Dysfunction Induced by α-Synuclein Accumulation in Parkinson's Disease. [PDF]

, 2013
Alpha-synuclein (α-Syn) accumulation/aggregation and mitochondrial dysfunction play prominent roles in the pathology of Parkinson's disease. We have previously shown that postmortem human dopaminergic neurons from PD brains accumulate high levels of ...
Adame, Anthony, Mueller, Sarina, Bender Andreas, Sarina Mueller, Koob Andrew O., Elstner Matthias, Elstner, Matthias, Desplats, Paula, Mante, Michael, Eliezer Masliah, Desplats Paula, Mueller Sarina, Spencer, Brian, Matthias Elstner, Thomas Klopstock, Masliah, Eliezer, Koob, Andrew O., Andreas Bender, Bender, Andreas, Masliah Eliezer, Anthony Adame, Klopstock Thomas, Rockenstein, Edward, Laub Christoph, Rockenstein Edward, Pham Emily, Brian Spencer, Mante Michael, Michael Mante, Klopstock, Thomas, Christoph Laub, Spencer Brian, Adame Anthony, Andrew O Koob, Paula Desplats, Emily Pham, Laub, Christoph, Pham, Emily, Edward Rockenstein   +38 more
core   +1 more source

Clinically translatable mitochondrial gene therapy in muscle using tandem mtZFN architecture

EMBO Molecular Medicine
Mutations in the mitochondrial genome (mtDNA) often lead to clinical pathologies. Mitochondrially-targeted zinc finger nucleases (mtZFNs) have been successful in reducing the levels of mutation-bearing mtDNA both in vivo and in vitro, resulting in a ...
Pavel A Nash, Keira M Turner, Christopher A Powell, Lindsey Van Haute, Pedro Silva-Pinheiro, Felix Bubeck, Ellen Wiedtke, Eloïse Marques, Dylan G Ryan, Dirk Grimm, Payam A Gammage, Michal Minczuk   +11 more
doaj   +1 more source

Actinomycin V Induces Apoptosis Associated with Mitochondrial and PI3K/AKT Pathways in Human CRC Cells

Marine Drugs, 2021
Actinomycin (Act) V, an analogue of Act D, presented stronger antitumor activity and less hepatorenal toxicity than Act D in our previous studies, which is worthy of further investigation.
Shiqing Jiang, E Zhang, Hang Ruan, Jiahui Ma, Xingming Zhao, Yaoyao Zhu, Xiaoyu Xie, Ningning Han, Jianjiang Li, Hao Zhang, Weidong Xie, Xia Li   +11 more
doaj   +1 more source

Structural insights and therapeutic targets in Acinetobacter baumannii capsule biosynthesis

FEBS Letters, EarlyView.
Hypervirulent KL49 A. baumannii's capsular polysaccharide contains the nonulosonic acid 8‐epi‐Leg5,7Ac2, synthesized by epimerization via ElaA, ElaB, and ElaC. Crystal structures of ElaA, ElaB, and ElaC reveal their role in CMP‐Leg5,7Ac2 synthesis and regioselective C8 epimerization.
Woo Cheol Lee, Junho Jeong, Chae Yeong Lee, Hyunjoon Cho, Man Hwan Oh, Je Chul Lee, Jee‐Young Lee, Yangmee Kim   +7 more
wiley   +1 more source

Mitochondrial Dynamics and Mitochondrial Dysfunction in Diabetes.

Acta medica Okayama, 2016
The mitochondria are involved in active and dynamic processes, such as mitochondrial biogenesis, fission, fusion and mitophagy to maintain mitochondrial and cellular functions. In obesity and type 2 diabetes, impaired oxidation, reduced mitochondrial contents, lowered rates of oxidative phosphorylation and excessive reactive oxygen species (ROS ...
Wada, Jun, Nakatsuka, Atsuko
openaire   +3 more sources

MOM19, an import receptor for mitochondrial precursor proteins [PDF]

, 1989
We have identified a 19 kd protein of the mitochondrial outer membrane (MOM19). Monospecific IgG and Fab fragments directed against MOM19 inhibit import of precursor proteins destined for the various mitochondrial subcompartments, including porin ...
Söllner, Thomas, Pfaller, Rupert, Pfanner, Nikolaus, Neupert, Walter, Griffiths, Gareth   +4 more
core   +1 more source