Results 71 to 80 of about 14,571 (197)

Deficiency of Mitochondrial Fatty Acid Enzyme, CPT1A, Underlies Airway Epithelial Barrier Dysfunction in Severe Asthma

Allergy, EarlyView.
Mitochondrial fatty acid enzyme, CPT1A, is deficient in the airway epithelium of severe asthma patients. Restoration of CPT1A expression improved epithelial barrier integrity via increasing mitochondrial respiration and ATP production in airway epithelial cells. Restoration of deficient epithelial CPT1A may represent a new treatment approach for severe
Muyun Wang, Haiyang Hu, Kun Wang, Di Wu, Ximing Liao, Jing Gao, Tian Li, Wujian Xu, Shaoyong Gao, Qiang Li, Pankaj Kumar Bhavsar, Wei Gao, Kian Fan Chung   +12 more
wiley   +1 more source

Diagnostic Yield and Clinical Impact of Comprehensive WES/WGS Testing Beyond Common Genetic Causes in Hereditary Optic Atrophy

Clinical Genetics, EarlyView.
Opticus atrophy—Genetic testing with WES/WGS in 62 patients with optic atrophy provided a genetic diagnosis in 21 patients (33.9%). 42.9% of these involved non‐OPA1 genes, including WFS1, ACO2, NR2F1, UCHL1, CACNA1F, and COQ2, where the genetic diagnosis prompted additional clinical evaluation, surveillance, or therapeutic intervention.
Katrine M. Johannesen, Karen Grønskov, Line Kessel, Sarah Linea von Holstein, Lisbeth Birk Møller, Mette Kjøbæk Gundestrup Andersen, Marianne Søndergaard Khinchi, Steffen Hamann, Marianne Wegener, Mette Bertelsen   +9 more
wiley   +1 more source

Unveiling Gut Homeostasis Disruption in Sepsis: Towards an Integrated Mechanistic and Translational Roadmap

Cell Proliferation, EarlyView.
Elucidating the contribution of gut‐organ axes will provide new insights for developing combined therapeutic strategies against sepsis‐associated multiple organ dysfunction. ABSTRACT Sepsis, a life‐threatening clinical syndrome precipitated by a maladaptive host response to infection, is associated with substantial morbidity and high mortality rates ...
Yichen Bao, Lin Qi, Guijun Zou, Xingpeng Yang, Yizhao Ma, Zhifu Li, Xiaohui Du, Pengyue Zhao   +7 more
wiley   +1 more source

Preclinical evaluation of candidate “kill or cure” strategies to treat MFN2-related lipodystrophy

Molecular Medicine
Background The mitofusin 2 (MFN2) R707W mutation causes debilitating human lipodystrophy featuring lower body adipose loss, upper body adipose hyperplasia, and dyslipidaemic insulin resistance.
Ineke Luijten, Xiong Weng, Ula Kibildyte, Jana Buchan, Ami Onishi, Jake Mann, Eleanor McKay, David Savage, Robert K. Semple   +8 more
doaj   +1 more source

ER proteostasis meets mitochondrial function: contact sites as hubs of communication and therapeutic targets

The FEBS Journal, EarlyView.
Proteostasis ensures proper protein folding, modification, and degradation, while its impairment triggers ER stress. Chronic ER stress and maladaptive UPR via the CHOP–ERO1 axis remodel ERMCs, altering calcium signaling and mitochondrial metabolism.
Giorgia Maria Renna, Alessandro Cherubini, Ersilia Varone, Serena Germani, Alice Marrazza, Ester Zito   +5 more
wiley   +1 more source

Ubiquitin and ubiquitin‐like modifications in the endoplasmic reticulum stress response

The FEBS Journal, EarlyView.
Endoplasmic reticulum (ER) stress activates various proteostasis control processes, including the unfolded protein response, ribosome‐associated quality control, and ER‐associated degradation. Ubiquitin and ubiquitin‐like modifications dynamically regulate these processes to determine cell fate, promoting adaptation or inducing cell death.
Tony Avril, Matthieu Le Gallo, Elodie Lafont   +2 more
wiley   +1 more source

One day of environment‐induced heat stress causes injury to the murine kidney

Experimental Physiology, EarlyView.
Abstract Environment‐induced heat stress (EIHS) results from sustained body temperature elevation owing to prolonged exposure to heat and humidity. We hypothesized that EIHS would cause kidney injury and cellular dysfunction. To test this hypothesis, female C57 mice were exposed to EIHS (n = 14; 37.6°C, 42.0% relative humidity) or thermoneutral (TN ...
Melissa Roths, Alyona Michael, Zachary J. Schlader, Joshua T. Selsby   +3 more
wiley   +1 more source

Mitochondrial alterations as potential early events in neuromuscular junction remodelling with muscle disuse

Experimental Physiology, EarlyView.
Abstract Physical inactivity impairs neuromuscular health, promoting skeletal muscle atrophy, mitochondrial changes, and neuromuscular junction (NMJ) instability. However, the interplay between mitochondria and NMJ alterations in the context of muscle disuse remains underexplored.
Evgeniia Motanova, Evgenii Lysenko, Fabio Sarto, Mladen Gasparini, Boštjan Šimunic, Rado Pišot, Marco V. Narici   +6 more
wiley   +1 more source

Mitochondrial capacities and quality control following short‐ and long‐term weight restoration after simulated anorexia nervosa

Experimental Physiology, EarlyView.
Abstract Anorexia nervosa (AN) is a psychiatric disorder characterized by prolonged caloric restriction and skeletal muscle atrophy. Mitochondrial health is a key mediator of muscle function, yet the role of mitochondria during AN and following weight regain has not been investigated. The objective of this study was to evaluate mitochondrial capacities
Megan E. Rosa‐Caldwell, Toby L. Chambers, Lauren Breithaupt, Ruqaiza Muhyudin, Patience Salvalina Okoto, Sadie R. Thompson, Emily E. Rothacker, Claire Greenhill, Katie A. Wood, Kevin A. Murach, Sarah H. White‐Springer, Ursula B. Kaiser, Seward B. Rutkove   +12 more
wiley   +1 more source

MicroRNA-214 Affects Fibroblast Differentiation of Adipose-Derived Mesenchymal Stem Cells by Targeting Mitofusin-2 during Pelvic Floor Dysfunction in SD Rats with Birth Trauma

Cellular Physiology and Biochemistry, 2017
Background/Aims: This study investigated whether microRNA-214 (miR-214) targets mitofusin-2 (Mfn2) in the process of fibroblast differentiation of adipose-derived mesenchymal stem cells (ADMSCs) during pelvic floor dysfunction (PFD) in Sprague Dawley (SD)
Jing Wu, Jiang Li, Wei-Kang Chen, Shang Liu, Jian-He Liu, Jing-Song Zhang, Ke-Wei Fang   +6 more
doaj   +1 more source