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Glycogen storage disease type II (GSDII), or Pompe disease, is a rare autosomal recessive metabolic disorder characterized by the deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). GAA deficiency results in the progressive accumulation of glycogen in cardiac and skeletal muscle tissues, leading to cellular dysfunction and clinical manifestations, including muscle weakness, respiratory difficulties, and cardiomyopathy. In this study, we report the derivation of three induced pluripotent stem cell (iPSC) lines from peripheral blood mononuclear cells of GSDII patients. These patient-derived iPSC lines can be used to study pathological features of the disease and evaluate therapeutic strategies for Pompe disease.
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http://dx.doi.org/10.1016/j.scr.2025.103823 | DOI Listing |
Eur J Pharmacol
September 2025
Eisai, Inc., 200 Metro Blvd. Nutley, NJ 07110, USA. Electronic address:
Phosphodiesterase 9 (PDE9) is an enzyme that hydrolyzes cyclic guanosine monophosphate (cGMP)-a second messenger that regulates neuronal plasticity and memory function. PDE9 inhibition has been shown to enhance cognitive function in rodents, underlining the potential of PDE9 inhibitors (PDE9Is) as novel therapeutics for cognitive dysfunction. Considering the critical role of nitric oxide (NO)-cGMP signaling cascade in acetylcholine (ACh) release, the combination of PDE9Is and acetylcholinesterase inhibitors may synergistically elevate ACh levels in the brain.
View Article and Find Full Text PDFBiochem Biophys Res Commun
September 2025
CERVO Brain Research Centre, Quebec City, QC, Canada; Department of Medicine, Faculty of Medicine, Université Laval, Quebec City, QC, Canada. Electronic address:
Brugada syndrome is a rare inherited cardiac arrhythmia disorder primarily characterized by ventricular fibrillation, which can lead to sudden cardiac death. It follows an autosomal dominant pattern of inheritance and is most associated with dysfunction of the cardiac sodium channel Nav1.5.
View Article and Find Full Text PDFInt J Artif Organs
September 2025
Department of Clinical Engineering, Faculty of Medical Science and Technology, Gunma Paz University, Takasaki-shi, Gunma, Japan.
Cardiovascular disease (CVD) is a leading cause of death worldwide. CVD includes conditions such as myocardial infarction (MI), arrhythmias, valvular heart disease, and cardiomyopathy. The limitations of heart treatment are related to the inability of damaged cells to regenerate, which leads to an increasing demand for new therapies.
View Article and Find Full Text PDFLife Sci Alliance
November 2025
Department of Viroscience, Erasmus MC, Rotterdam, The Netherlands
Enterovirus D68 (EV-D68) is an emerging respiratory virus associated with extra-respiratory complications, especially acute flaccid myelitis. However, the pathogenesis of acute flaccid myelitis is not fully understood. It is hypothesised that through infection of skeletal muscles, the virus further infects motor neurons via the neuromuscular junction.
View Article and Find Full Text PDFLife Sci Alliance
November 2025
Immunoregulation Research Group, Max Planck Institute of Biochemistry, Martinsried, Germany
Amino acid (AA) detection is fundamental for cellular function, balancing translation demands, biochemical pathways, and signaling networks. Although the GCN2 and mTORC1 pathways are known to regulate AA sensing, the global cellular response to AA deprivation remains poorly understood, particularly in non-transformed cells, which may exhibit distinct adaptive strategies compared with cancer cells. Here, we employed murine pluripotent embryonic stem (ES) cells as a model system to dissect responses to AA stress.
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