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Skeletal progenitor LRP1 deficiency causes severe and persistent skeletal defects with Wnt pathway dysregulation.
Bone Res
January 2025
Institute of Life Course and Medical Sciences, Faculty of Health and Life Sciences, University of Liverpool, Liverpool, UK.
Low-density lipoprotein receptor-related protein 1 (LRP1) is a multifunctional endocytic receptor whose dysfunction is linked to developmental dysplasia of the hip, osteoporosis and osteoarthritis. Our work addresses the critical question of how these skeletal pathologies emerge. Here, we show the abundant expression of LRP1 in skeletal progenitor cells at mouse embryonic stage E10.
View Article and Find Full Text PDFTroponins and Skeletal Muscle Pathologies.
Biochemistry (Mosc)
December 2024
Faculty of Biology, Lomonosov Moscow State University, Moscow, 119234, Russia.
Skeletal muscles account for ~30-40% of the total weight of human body and are responsible for its most important functions, including movement, respiration, thermogenesis, and glucose and protein metabolism. Skeletal muscle damage negatively impacts the whole-body functioning, leading to deterioration of the quality of life and, in severe cases, death. Therefore, timely diagnosis and therapy for skeletal muscle dysfunction are important goals of modern medicine.
View Article and Find Full Text PDFThe novel use of the CFTR corrector C17 in muscular dystrophy: Pharmacological profile and in vivo efficacy.
Biochem Pharmacol
January 2025
Department of Biomedical Sciences, University of Padova, 35131 Padova, Italy,. Electronic address:
Sarcoglycanopathies are rare forms of severe muscular dystrophies currently without a therapy. Mutations in sarcoglycan (SG) genes cause the reduction or absence of the SG-complex, a tetramer located in the sarcolemma that plays a protective role during muscle contraction. Missense mutations in SGCA, which cause α-sarcoglycanopathy, otherwise known as LGMD2D/R3, lead to folding defective forms of α-SG that are discarded by the cell quality control.
View Article and Find Full Text PDFNext-generation sequencing analysis of bacterial species present in the sequestrum of medication-related osteonecrosis of the jaw patients.
Arch Oral Biol
January 2025
Department of Oral and Maxillofacial Surgery, Pusan National University, Yangsan, South Korea; Dental and Life Science Institute, School of Dentistry, Pusan National University, Yangsan, South Korea; Dental Research Institute, Pusan National University Dental Hospital, Yangsan, South Korea. Electronic address:
Objective: Antiresorptives cases of adverse reactions to highly effective drugs used to treat skeletal-related events that occur during treatment of osteoporosis or malignancy have been reported in the jaw. To date, there is no clear pathophysiology for medication-related osteonecrosis of the jaw (MRONJ), but the role of inflammation or infection has been proposed as one of several possible pathogenesis theories. This study was designed to investigate the role of infection in the pathogenesis of MRONJ by analyzing the microbial communities observed in the bone samples from MRONJ patients.
View Article and Find Full Text PDFPreoperative myosteatosis and perioperative serum chloride levels predict 180 day major complications after radical cystectomy.
Sci Rep
January 2025
Division of Urology, Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
This study aimed to investigate the role of myosteatosis, sarcopenia, and perioperative serum biomarkers as independent predictors of major complications within 180 days following radical cystectomy (RC) for muscle-invasive bladder cancer (MIBC). We retrospectively analyzed of 127 MIBC patients who underwent RC between 2013 and 2023 at a single institution. Preoperative body composition was assessed using CT scans at the L3 vertebral level to measure psoas muscle density (PMD), skeletal muscle density (SMD), axial muscle density (AMD), and muscle indices.
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