Results 11 to 20 of about 13,400,035 (354)
CFTR activity and mitochondrial function
Redox Biology, 2013 Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Angel Gabriel Valdivieso +1 more
doaj +4 more sources
Evaluating the mitochondrial function [PDF]
Diabetology & Metabolic Syndrome, 2012 We have read the article by Xia et.al. entitled as "L-carnitine ameliorated fatty liver in high-calorie diet/STZ induced type 2 diabetic mice by improving mitochondrial function" with great interest. In this article, the authors concluded that L-carnitine might be an effective liver cell protector for delaying the progression of type 2 diabetes ...
Akgul Emin +2 more
doaj +3 more sources
Mitochondrial Functioning ≠ General Intelligence [PDF]
Journal of Intelligence, 2020 Geary puts forward an appealing argument for the consideration of mitochondrial functioning as a candidate for a formative g Geary (2019); it is also an ambitious argument [...]
Alexander O. Savi +3 more
openaire +5 more sources
Mitochondrial Function and Dysfunction in Dilated Cardiomyopathy
Frontiers in Cell and Developmental Biology, 2021 Cardiac tissue requires a persistent production of energy in order to exert its pumping function. Therefore, the maintenance of this function relies on mitochondria that represent the “powerhouse” of all cardiac activities.
D. Ramaccini +10 more
semanticscholar +1 more source
The Impact of Mitochondrial Deficiencies in Neuromuscular Diseases
Antioxidants, 2020 Neuromuscular diseases (NMDs) are a heterogeneous group of acquired or inherited rare disorders caused by injury or dysfunction of the anterior horn cells of the spinal cord (lower motor neurons), peripheral nerves, neuromuscular junctions, or skeletal ...
Judith Cantó-Santos +2 more
doaj +1 more source
Mitochondrial function in development and disease
Disease Models & Mechanisms, 2021 Mitochondria are organelles with vital functions in almost all eukaryotic cells. Often described as the cellular ‘powerhouses’ due to their essential role in aerobic oxidative phosphorylation, mitochondria perform many other essential functions beyond ...
M. Rossmann +3 more
semanticscholar +1 more source
Relevance of sex-differenced analyses in bioenergetics and nutritional studies
Frontiers in Nutrition, 2022 Sex-biased analyses still remain as one of the biggest limitations to obtain universal conclusions. In biomedicine, the majority of experimental analyses and a significant amount of patient-derived cohort studies exclusively included males.
Glòria Garrabou +7 more
doaj +1 more source
Mitochondrial function requires NGLY1 [PDF]
Mitochondrion, 2018 Mitochondrial respiratory chain (RC) diseases and congenital disorders of glycosylation (CDG) share extensive clinical overlap but are considered to have distinct cellular pathophysiology. Here, we demonstrate that an essential physiologic connection exists between cellular N-linked deglycosylation capacity and mitochondrial function.
Jianping, Kong +8 more
openaire +2 more sources
Unravelling inclusion body myositis using a patient‐derived fibroblast model
Journal of Cachexia, Sarcopenia and Muscle, 2023 Background Inclusion body myositis (IBM) is an inflammatory myopathy clinically characterized by proximal and distal muscle weakness, with inflammatory infiltrates, rimmed vacuoles and mitochondrial changes in muscle histopathology.
Judith Cantó‐Santos +18 more
doaj +1 more source
Antioxidants, 2022 The quantification of mitochondrial respiratory chain (MRC) enzymatic activities is essential for diagnosis of a wide range of mitochondrial diseases, ranging from inherited defects to secondary dysfunctions.
Nuria Bujan +18 more
doaj +1 more source