Results 71 to 80 of about 304,862 (304)

Impact of C4BPA on Muscle progenitor cell differentiation: insights for Duchenne muscular dystrophy treatment

Cell Death and Disease
Fibroadipogenic precursor cells (FAPs) are key contributors to the fibrotic and adipogenic remodeling observed in Duchenne muscular dystrophy (DMD), yet their precise role in muscle degeneration remains unclear.
Esther Fernández-Simón, Ainoa Tejedera-Villafranca, Xiomara Fernández-Garabay, James Clark, Panagiotis Katsikis, Priyanka Mehra, Andrew Galloway, Alexandra Monceau, Elisa Villalobos, Dan Cox, Javier Ramón-Azcón, Juan M. Fernández-Costa, Jordi Díaz-Manera   +12 more
doaj   +1 more source

Early Retinal UCHL1 Dysregulation Coupled With Synaptic Loss Reflects Alzheimer's Disease Severity

Advanced Science, EarlyView.
This study identifies synapse‐enriched deubiquitinase UCHL1 as an early Aβ‐responsive regulator of retinal synaptopathy in Alzheimer's disease. Retinal UCHL1 loss accompanies excitatory synapse degeneration, p75NTR activation, and neuroinflammation, and predicts Braak stage and cognitive decline. Aβ42 fibrils trigger synaptic and UCHL1 depletion before
Altan Rentsendorj, Jean‐Philippe Vit, Alexandre Hutton, Yosef Koronyo, Saba Shahin, Edward Robinson, Ernesto Barron, Anthony Rodriguez, Ousman Jallow, Julia Sheyn, Bhakta Prasad Gaire, Lalita Subedi, Miyah R. Davis, Barathan Gnanabharathi, Alexander V. Ljubimov, Stuart L. Graham, Vivek K. Gupta, Mehdi Mirzaei, Debra Hawes, Katlin Silm, Keith L. Black, Lon S. Schneider, Alfredo A. Sadun, Jesse G. Meyer, Dieu‐Trang Fuchs, Maya Koronyo‐Hamaoui   +25 more
wiley   +1 more source

Saponins as Natural Adjuvant for Antisense Morpholino Oligonucleotides Delivery In Vitro and in mdx Mice

Molecular Therapy: Nucleic Acids, 2018
Antisense oligonucleotide (AON) therapy for Duchenne muscular dystrophy has drawn great attention in preclinical and clinical trials, but its therapeutic applications are still limited due to inefficient delivery.
Mingxing Wang, Bo Wu, Sapana N. Shah, Peijuan Lu, Qilong Lu   +4 more
doaj   +1 more source

HMGCR‐Driven Cholesterol Metabolism Promotes Osteoarthritis Progression by Accelerating Synovial Fibroblast Senescence

Advanced Science, EarlyView.
In the pathological context of osteoarthritis (OA), the phosphorylation of AKT1 at Ser473 enhances its binding to Lys140 of Insig1, which facilitates the formation of AKT1–Insig1 complex. Subsequently, the activation of AKT1 promotes the phosphorylation of Insig1 at Ser189, potentially enhancing the dissociation of Insig1 from sterol regulatory element‑
Xiaoqi Zhang, Siru Zhou, Xi Liu, Wei Xiang, Xiuqin Peng, Yu Tian, Song Li, Hailin Wang, Bo Liao, Shunzheng Fang, Runze Jin, Jinhui Wu, Chuanqing Bai, Wei Zhang, Bo Huang, Bingfei Li, Lifeng Chen, Lin Chen, Bin Zhang, Zhenhong Ni   +19 more
wiley   +1 more source

Priorities when deciding on participation in early-phase gene therapy trials for Duchenne muscular dystrophy: a best–worst scaling experiment in caregivers and adult patients

Orphanet Journal of Rare Diseases, 2019
Purpose Several gene therapy trials for Duchenne muscular dystrophy initiated in 2018. Trial decision making is complicated by non-curative, time-limited benefits; the progressive, fatal course; and high unmet needs.
Ryan S. Paquin, Ryan Fischer, Carol Mansfield, Brennan Mange, Katherine Beaverson, Annie Ganot, Amy Strong Martin, Carl Morris, Colin Rensch, Valeria Ricotti, Leo J. Russo, Alesia Sadosky, Edward C. Smith, Holly L. Peay   +13 more
doaj   +1 more source

Maternal Exercise Rescues Embryonic Osteogenesis Impaired due to POLG Mutation Through a Potential Apelin‐ATF4 Axis

Advanced Science, EarlyView.
Maternal exercise (ME) increases apelin abundance across maternal and fetal tissues and is associated with improved fetal osteogenesis under POLG mutation‐induced mitochondrial dysfunction. Apelin‐APJ signaling is linked to enhanced mitochondrial function, Akt phosphorylation, and ATF4‐RUNX2 association, supporting coordinated fetal bone remodeling ...
Song Ah Chae, Ryan C. Riddle, Zhihua Jiang, Chaeyoung Shin, Min Du, Jun Seok Son   +5 more
wiley   +1 more source

Lipids of dystrophic and normal mouse muscle: whole tissue and particulate fractions

Journal of Lipid Research, 1970
Myofibrillar, mitochondrial, and microsomal fractions were prepared from normal and dystrophic mouse limb muscle by differential centrifugation and analyzed for phospholipids and cholesterol.
K. OWENS, B.P. HUGHES
doaj   +1 more source

Muscle strength deficiency and mitochondrial dysfunction in a muscular dystrophy model of C. elegans and its functional response to drugs [PDF]

, 2018
Muscle strength is a key clinical parameter used to monitor the progression of human muscular dystrophies including Duchenne and Becker muscular dystrophies.
Pollard, Amelia K., Gaffney, CJ, Szewczyk, Nathaniel J., Hewitt, Jennifer E., Lesanpezeshki, Leila, Gaffney, Christopher J., Leila Lesanpezeshki, Vanapalli, Siva A., Pollard, A.K., Hewitt, Jennifer, Amelia K. Pollard, Siva A. Vanapalli, Gaffney, C.J., Szewczyk, N.J., Vanapalli, S.A., Deane, CS, Deane, C.S., Lesanpezeshki, L., Vanapalli, Siva, Timothy Etheridge, Gaffney, Christopher James, Szewczyk, Nathaniel, Etheridge, T, Vanapalli, SA, Jennifer E. Hewitt, Hewitt, J.E., Szewczyk, NJ, Etheridge, Timothy, Colleen S. Deane, Deane, Colleen S., Nathaniel J. Szewczyk, Lesanpezeshki, L, Etheridge, T., Pollard, Amelia, Hewitt, JE, Deane, Colleen, Christopher J. Gaffney, Pollard, AK   +37 more
core   +1 more source

Glucocorticoid Receptor Signaling in Myeloid Cells Orchestrates Inflammation Resolution and Muscle Repair

Advanced Science, EarlyView.
Glucocorticoids (GC) are widely used to reduce inflammation. We show that the glucocorticoid receptor in myeloid cells regulates macrophage cell cycle and genome integrity during muscle regeneration. We demonstrate that dexamethasone administration during the early inflammatory phase delays muscle repair by increasing macrophage proliferation ...
Sirine Souali‐Crespo, Joe G. Rizk, Emilia Calvano, Rajesh Sahu, Emina Colovic, Imane Chabba, Valentine Gilbart, Erwan Grandgirard, Bastien Morlet, Qingshuang Cai, Daniel Metzger, Delphine Duteil   +11 more
wiley   +1 more source

Duchenne muscular dystrophy : mutation profiling in view of the emerging gene-based therapies [PDF]

, 2010
Includes bibliographical references (leaves 97-115).Duchenne Muscular Dystrophy (DMD) is a lethal, X-linked, recessive muscle-wasting disorder affecting 1 in 3 500 live male births worldwide, for which only palliative care is available to date.
Esterhuizen, Alina
core   +1 more source