Results 61 to 70 of about 119,249 (261)

ALDOA Promotes Glycolysis and NLRP3/GSDMD Pyroptosis to Accelerate ALS Progression

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is characterized by progressive motor neuron degeneration. Glycolytic dysregulation is implicated in disease progression, yet the underlying mechanisms remain unclear. This study investigates how Aldolase A (ALDOA) drives ALS progression through glycolysis‐mediated motor neuron pyroptosis.
Kaixin Yan, Yan Jiang, Yuxuan Yong, Tianshuo Zhang, Niannian Zhang, Qianqian Zeng, Xue Gong, Li Meng, Fangfang Bi, Yongmin Liu   +9 more
wiley   +1 more source

RNA Sequencing Resolves Cryptic Pathogenic Variants in Mitochondrial Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Mitochondrial diseases are the most common inherited metabolic disorders, characterized by pronounced clinical and genetic heterogeneity that complicates molecular diagnosis. Although DNA‐based sequencing approaches have become standard in genetic testing, up to half of patients remain without a definitive diagnosis.
Zhimei Liu, Xin Duan, Fatemeh Peymani, Jia Wang, Chengjia Bao, Chaolong Xu, Ying Zou, Zixuan Zhang, Yunxi Zhang, Tongyue Li, Martin Pavlov, Junling Wang, Minhan Song, Tianyu Song, Xiaodi Han, Mingxi Sun, Danmin Shen, Ruoyu Duan, Huafang Jiang, Manting Xu, Holger Prokisch, Fang Fang   +21 more
wiley   +1 more source

Longitudinal Assessment of Biomarkers in ALS: Discriminative Biomarkers for Disease Progression and Survival

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To assess the association and discriminative performance of serum biomarkers with clinical disease progression and survival in patients with amyotrophic lateral sclerosis (ALS). Methods This retrospective study, conducted at Houston Methodist Hospital, Houston, TX, used longitudinal serum samples collected between January 2018 and ...
David R. Beers, Yueh‐Yun Lin, Jason R. Thonhoff, Aaron D. Thome, Alireza Faridar, Weihua Zhao, Shixiang Wen, Stanley H. Appel   +7 more
wiley   +1 more source

A 17 Year Old With Developmental Delay Presenting With Increasing Confusion and Imbalance

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Methylmalonic acidemia is an autosomal recessive genetic disorder primarily caused by defects in methylmalonyl‐CoA mutase and cobalamin (vitamin B12) metabolism. These defects disrupt the tricarboxylic acid cycle and oxidative phosphorylation, leading to the abnormal accumulation of metabolic products such as methylmalonic acid, propionic acid,
Wei Zhao, Yingli Zhang, Hongliang Zheng
wiley   +1 more source

Heightened mitochondrial respiration in CF cells is normalised by triple CFTR modulator therapy through mechanisms involving calcium

Heliyon
Background: Cystic fibrosis (CF) is associated with increased resting energy expenditure. However, the introduction of elexacaftor/tezacaftor/ivacaftor (ETI) has resulted in a paradigm shift in nutritional status for many people with CF, with increase ...
H.H. Jarosz-Griffiths, L.R. Caley, S. Lara-Reyna, S. Savic, I.J. Clifton, M.F. McDermott, D.G. Peckham   +6 more
doaj   +1 more source

A Systematic Study of GelMA‐Carbopol Bioinks for High‐Fidelity Extrusion 3D Bioprinting at Physiological Temperatures

Advanced Healthcare Materials, EarlyView.
Gonzalez Martinez and collaborators develop a strategy to formulate high performance GelMA‐based bioinks with low solids contents. The resulting bioinks enable 3D bioprinting at 37 °C of high‐fidelity structures with tunable mechanical properties that support high cell viability and function.
David A. González‐Martínez, Aidee V. Arizpe Tafoya, Eduardo González‐Martínez, Mabel Barreiro Carpio, Laura Daniela Serrano Andrade, Christopher T. N. Chow, Mohammadhossein Dabaghi, Jeremy A. Hirota, Jose M. Moran‐Mirabal   +8 more
wiley   +1 more source

CPP–PEG‐Guided Surface Engineering of Mitochondria Enables Efficient Cellular Uptake and Respiratory Modulation

Advanced Materials Interfaces, EarlyView.
PEG‐guided mitochondrial surface engineering supports structural stabilization and controlled presentation of cell‐penetrating peptides. CPP–PEG‐modified mitochondria exhibit enhanced cellular uptake and uptake‐associated respiratory modulation, providing a proof‐of‐concept framework for modular organelle engineering and future bioenergetic modulation ...
Masahiro Shiraishi, Yukari Muramatsu, Akihiro Nakaya, Yuji Maruo, Rick C. Tsai, Masashi Suganuma, Hisashi Ohta, Atsuhito Takeda, Hideyoshi Harashima, Yuma Yamada   +9 more
wiley   +1 more source

Human tissue-specific MSCs demonstrate differential mitochondria transfer abilities that may determine their regenerative abilities

Stem Cell Research & Therapy, 2018
Background Recent studies have demonstrated mesenchymal stem cells (MSCs) as effective mitochondrial donors with therapeutic success in multiple experimental models of human disease.
Swati Paliwal, Rituparna Chaudhuri, Anurag Agrawal, Sujata Mohanty   +3 more
doaj   +1 more source

Malectin Alleviates Endoplasmic Reticulum Stress in Gestational Diabetes Mellitus via Glycoprotein Quality Control Mechanisms

Advanced Science, EarlyView.
Malectin alleviates high glucose‐induced ER stress and damage in placental trophoblasts, a function dependent on its six critical carbohydrate‐binding residues. In a GDM mouse model, administration of TAT‐Malectin ameliorated hyperglycemia and placental ER stress and prevented fetal macrosomia.
Jiahui Zhu, Yumeng Zhang, Ye Wang, Xiaoyu Zhu, Ailin Yuan, Weijing Yin, Huangmin Yu, Xuemin Pang, Yufeng He, Yuchen Wang, Tong Wang, Yong Li, Yunlong Si   +12 more
wiley   +1 more source

Nitric Oxide Does Not Improve Liver Mitochondrial Function 48 Hours After Cecal Ligation and Perforation in Experimental Sepsis

Antioxidants
Nitric oxide (NO) has a dual effect on mitochondria. Incubating liver mitochondria with NO improves oxidative phosphorylation (OXPHOS) efficiency by decreasing state 4 respiration more than ATP synthesis and preventing mitochondrial permeability ...
Pierre Eyenga, Shey-Shing Sheu
doaj   +1 more source