The essential role of fructose-1,6-bisphosphatase 2 enzyme in thermal homeostasis upon cold stress
Body temperature: Enzyme critical to heat production in muscle When simple sugars in the diet are scarce, skeletal muscle can still generate heat under cold conditions thanks to an enzyme that converts a metabolic byproduct into complex carbohydrates.
Hyun-Jun Park+5 more
doaj +1 more source
Mitochondrial networks are defined as a continuous matrix lumen, but the morphological feature of neuronal mitochondrial networks is not clear due to the lack of suitable analysis techniques.
Shu-Jiao Li+12 more
doaj +1 more source
Fingolimod phosphate protection against mitochondrial damage in neuronal cells [PDF]
Background: Major role of oxidative stress in the pathogenesis of neurodegenerative diseases have been suggested, being mitochondria one of the main sources of ROS. Aim: In the present work, we have studied the antioxidant effect of fingolimod phosphate
Alvarez-Gil, Antonio+8 more
core +1 more source
Mitochondria contain the respiratory chain enzyme complexes that carry out oxidative phosphorylation and produce the main part of cellular energy in the form of ATP. Although several proteins related with signalling, assembling, transporting, and enzymatic function can be impaired in mitochondrial diseases, most frequently the activity of the ...
openaire +2 more sources
Neuroimaging of mitochondrial disease [PDF]
Mitochondrial disease represents a heterogeneous group of genetic disorders that require a variety of diagnostic tests for proper determination. Neuroimaging may play a significant role in diagnosis. The various modalities of nuclear magnetic resonance imaging (MRI) allow for multiple independent detection procedures that can give important anatomical ...
Seth D. Friedman+2 more
openaire +3 more sources
Dynamin-related protein 1 is required for normal mitochondrial bioenergetic and synaptic function in CA1 hippocampal neurons. [PDF]
Disrupting particular mitochondrial fission and fusion proteins leads to the death of specific neuronal populations; however, the normal functions of mitochondrial fission in neurons are poorly understood, especially in vivo, which limits the ...
Berthet, A+12 more
core +3 more sources
A variable neurodegenerative phenotype with polymerase gamma mutation [PDF]
mtDNA replication and repair, causes mitochondrial diseases including autosomal dominant progressive external ophthalmoplegia (PEO),1 childhood hepato-encephalopathy (Alpers– Huttenlocher syndrome), adult-onset spinocerebellar ataxia, and sensory nerve
Baruffini, E.+8 more
core +1 more source
Clinically translatable mitochondrial gene therapy in muscle using tandem mtZFN architecture
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+11 more
doaj +1 more source
Recombinant Mitochondrial Transcription Factor A with N-terminal Mitochondrial Transduction Domain Increases Respiration and Mitochondrial Gene Expression in G11778A Leber's Hereditary Optic Neuropathy Cybrid Cells [PDF]
Diseases involving mitochondrial defects usually manifest themselves in high-energy, post-mitotic tissues such as brain, retina, skeletal and cardiac muscle and frequently cause deficiencies in mitochondrial bioenergetics.
Caitlin K. Quigley+5 more
core +1 more source
Metabolic dysfunction‐associated steatotic liver disease (MASLD) affects nearly one‐third of the global population and poses a significant risk of progression to cirrhosis or liver cancer. Here, we discuss the roles of hepatic dendritic cell subtypes in MASLD, highlighting their distinct contributions to disease initiation and progression, and their ...
Camilla Klaimi+3 more
wiley +1 more source