Results 61 to 70 of about 31,581 (280)

Differentiation of norm and pathology during selective biochemical skreening of lysosomal storage diseases with increased excretion of oligosaccharides [PDF]

The Ukrainian Biochemical Journal, 2015
Oligosaccharides are a class of polymeric carbohydrates, which are constituents of a glycoside portion of glycoprotein and glycolipid molecules. The lysosomal hydrolase dysfunction due to lysosomal storage disorders results in partial or complete failure
N. Y. Mytsyk, N. V. Olkhovych, N. G. Gorovenko   +2 more
doaj   +1 more source

Lilrb4a Suppression Reprograms Microglia to Mitigate APOE4‐Associated Amyloid Plaques and Cerebral Amyloid Angiopathy in Association With a PPAR‐Linked Pro‐Clearance State

Advanced Science, EarlyView.
Targeting Lilrb4a in Apolipoprotein E4 (APOE4)‐associated Alzheimer's disease (AD) reprograms microglia toward a beneficial, phagocytic state. Genetic deletion or antisense inhibition of Lilrb4a suppresses p‐SHP2/NF‐κB/STAT1 signaling, restores PPAR‐linked lipid and energy metabolism, and reduces amyloid plaque burden and cerebral amyloid angiopathy ...
Changxu Nie, Ruixi Yang, Xiaotong Wang, Ping Jia, Xueqi Zhang, Yaqi Dai, Xue Bai, Sijia Duan, Yufeng Li, Peng Zheng, Xin Tian, Li Jiang, Chao Wang   +12 more
wiley   +1 more source

The rapidly evolving view of lysosomal storage diseases

EMBO Molecular Medicine, 2021
Lysosomal storage diseases are a group of metabolic disorders caused by deficiencies of several components of lysosomal function. Most commonly affected are lysosomal hydrolases, which are involved in the breakdown and recycling of a variety of complex ...
Giancarlo Parenti, Diego L Medina, Andrea Ballabio   +2 more
doaj   +1 more source

Pathophysiology of neuropathic lysosomal storage disorders

, 2010
Although neurodegenerative diseases are most prevalent in the elderly, in rare cases, they can also affect children. Lysosomal storage diseases (LSDs) are a group of inherited metabolic neurodegenerative disorders due to deficiency of a specific protein ...
Cinzia Maria Bellettato, BELLETTATO CM, Maurizio Scarpa, SCARPA, MAURIZIO   +3 more
core   +1 more source

Dual Physiological Barriers Bypassed by a Silk‐Based Supramolecular Protein Delivery Platform for Neuroinflammation Mitigation in Alzheimer's Disease

Advanced Science, EarlyView.
A supramolecular nanocomplex based on phenolic‐modified silk sericin is developed to deliver antioxidant enzymes across the blood–brain barrier and escape lysosomal degradation. This dual‐barrier‐crossing system reduces neuroinflammation and improves cognitive performance in Alzheimer's disease mouse model, offering a promising strategy for protein ...
Doudou Hu, Tiandong Li, Jian He, Yujian Jiang, Yeyuan Wang, Jingchen Sun   +5 more
wiley   +1 more source

Lysosome

Journal of Inborn Errors of Metabolism and Screening, 2016
Since Christian de Duve first described the lysosome in the 1950s, it has been generally presented as a membrane-bound compartment containing acid hydrolases that enables the cell to degrade molecules without being digested by autolysis.
Ursula Matte BSc, PhD, Gabriela Pasqualim BSc, MSc   +1 more
doaj   +1 more source

ZDHHC18‐Mediated Palmitoylation of ORF3a Promotes SARS‐CoV‐2 Pathogenesis by Antagonizing TRIM16‐Mediated Ubiquitination and Proteasomal Degradation

Advanced Science, EarlyView.
Palmitoylation by ZDHHC18 blocks ORF3a K27‐linked ubiquitination mediated by TRIM16, thereby preventing its proteasomal degradation and strengthening viral pathogenesis. Targeting palmitoylation through a pharmacological inhibitor (2‐BP), a competitive inhibitory peptide (OPIP), or adenovirus‐mediated knockdown of ZDHHC18 expression presents a ...
Sidi Yang, Kun Li, Lihong Liu, Linsen Zeng, Qifeng Deng, Jiacheng Huang, Xiaoran Dong, Xin Wang, Jianwei Liang, Hongchao Liu, Hong Peng, Yuxin Lin, Xiaolu Xie, Yuzhen Ye, Tiefeng Xu, Zhaohuan Wang, Chun‐Mei Li, Deyin Guo   +17 more
wiley   +1 more source

Changes in Angiogenesis and Bone Turnover Markers in Patients with Gaucher Disease Developing Osteonecrosis

Metabolites
Background/Objectives: Patients with Gaucher disease have a high risk of bone disease, with osteonecrosis representing the most debilitating complication. The pathogenesis of osteonecrosis has not been fully elucidated yet, and there is an unmet need for
Simona D’Amore, Kenneth Eric Poole, Uma Ramaswami, Derralynn Hughes, Kathleen Page, Antonio Giovanni Solimando, Angelo Vacca, Timothy Martin Cox, Patrick Deegan   +8 more
doaj   +1 more source

Recent advances in gene therapy for lysosomal storage disorders

, 2015
David PW Rastall,1 Andrea Amalfitano1,2 1Department of Microbiology and Molecular Genetics, 2Department of Pediatrics, College of Osteopathic Medicine, Michigan State University, East Lansing, MI, USA Abstract: Lysosomal storage disorders (LSDs) are a ...
Amalfitano A, Rastall DP
core  

A Skull Bone Marrow‐to‐Brain Axis Links Osteoblastic Activity to Myeloid Cell Trafficking, Cerebral Blood Flow, and Cognition in Alzheimer's Progression

Advanced Science, EarlyView.
This study reveals that Alzheimer's disease–linked APP expression in bone‐forming cells drives skull bone marrow remodeling and alters its vascular connections to the brain. These changes disrupt immune cell trafficking, cerebral blood flow, and cognition. Targeting bone marrow macrophages restores brain function, highlighting a previously unrecognized
Lei Xiong, Dong Sun, Hao‐Han Guo, Daehoon Lee, Zhipeng Liu, Lin Mei, Wen‐Cheng Xiong   +6 more
wiley   +1 more source