Results 141 to 150 of about 2,687,044 (278)

Chlorination‐Driven BODIPY Fluorescent Probes for the Selective Monitoring of Myeloperoxidase Activity in Cells and Inflamed Mouse Models

Angewandte Chemie, EarlyView.
A meso‐carboxamide‐substituted BODIPY fluorescent probe reports myeloperoxidase (MPO) activity via electrophilic chlorination by MPO‐derived HOCl, generating a highly emissive chlorinated product with a ca. 40 nm bathochromic shift. Applications in quantitative MPO assays, cellular imaging of MPO‐derived HOCl, redox‐dependent discrimination of cancer ...
Siyoung Cho, Yongyang Luo, Jeehyeon Bae, Youngmi Kim   +3 more
wiley   +2 more sources

Mitochondrial Quality Control in Degenerative Musculoskeletal Diseases: Signaling Networks and Therapeutic Landscapes

Advanced Science, EarlyView.
Mitochondrial quality control (MQC) collapse redefines osteoarthritis, intervertebral disc degeneration, and osteoporosis as disorders of organelle dysfunction. Damaged mitochondria leak mtDNA and metabolites, driving cGAS‐STING inflammation, epigenetic reprogramming, and metabolic memory.
Haiyang Gao, Zhangrong Cheng, Xianglong Chen, Wenbo Wu, Wang Wu, Zimu Yu, Cao Yang, Yukun Zhang   +7 more
wiley   +1 more source

Miglustat in Neuronopathic Lysosomal Storage Disorders: Biological Rationale, Clinical Evidence, and Limits of Repurposing. [PDF]

Int J Mol Sci
Lipiński P, Węgrzyn G.
europepmc   +1 more source

Examining the Role of a Functional Deficiency of Iron in Lysosomal Storage Disorders with Translational Relevance to Alzheimer's Disease. [PDF]

Cells, 2023
LeVine SM.
europepmc   +1 more source

Sacral Neural Crest‐Derived Extracellular Vesicles Promote Pelvic Ganglion Repair Through Anti‐Pyroptotic Effects Involving ANXA1–FPR2 Signaling

Advanced Science, EarlyView.
Extracellular vesicles derived from human pluripotent stem cell‐derived sacral neural crest cells promote repair of injured major pelvic ganglia and improve colorectal function. These vesicles reduce oxidative stress and pyroptosis, with ANXA1–FPR2 signaling contributing to their protective effects, and show a favorable therapeutic profile compared ...
Ting Yang, Wenwei Liu, Hufei Wang, Yuxin Wu, Yuanchen Ma, Xiaofeng Wang, Tianxiang Lei, Qiao Tang, Yebohao Zhou, Weiqiang Li, Andy Peng Xiang, Wu Song   +11 more
wiley   +1 more source

Autophagy-Lysosomal Dysfunction as a Converging Mechanism of Cardiomyopathy in Lysosomal Storage Disorders: From Pathobiology to Targeted Therapy. [PDF]

Int J Mol Sci
Lee CL, Chuang CK, Chang YH, Chiu HC, Tu YR, Lo YT, Wu JY, Lin HY, Lin SP.   +8 more
europepmc   +1 more source

Facial features of lysosomal storage disorders. [PDF]

Expert Rev Endocrinol Metab, 2022
D'Souza A, Ryan E, Sidransky E.
europepmc   +1 more source

Characterizing enteric pathology in MPS IIIA mice suggests disease-specific vulnerability among lysosomal storage disorders. [PDF]

JCI Insight
Ziółkowska EA, Williams L, Eultgen E, Kick GR, Ding X, Sorensen A, Le S, Doray B, Dickson PI, Heuckeroth RO, Cooper JD.   +10 more
europepmc   +1 more source

Light and Shadows in Newborn Screening for Lysosomal Storage Disorders: Eight Years of Experience in Northeast Italy. [PDF]

Int J Neonatal Screen, 2023
Gragnaniello V, Cazzorla C, Gueraldi D, Puma A, Loro C, Porcù E, Stornaiuolo M, Miglioranza P, Salviati L, Burlina AP, Burlina AB.   +10 more
europepmc   +1 more source

Differential Diagnosis of Mucopolysaccharidoses Types I, II, III, IVA, and VI Through Analysis of Leukocytic Inclusions

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Mucopolysaccharidoses (MPS) are lysosomal storage disorders characterized by the accumulation of glycosaminoglycans (GAGs), which can lead to cytoplasmic alterations in leukocytes. The objective of this study was to characterize leukocyte inclusions in patients with different types of MPS and assess their diagnostic relevance.
Márcio A. W. Melo, Marcelo S. Kertenetzky, Cristina M. Silveira, Maíra M. Ribeiro, Paula F. Medeiros, Gabriela S. Arcanjo   +5 more
wiley   +1 more source