Results 111 to 120 of about 2,885,518 (281)

Magnetically Guided Macrophage Immunobots Coordinate Iron‐Metabolic Cascades and Immunogenic Ferroptosis for Tumor Immunotherapy

Advanced Science, EarlyView.
Magnetically guided macrophage immunobots integrate active tumor targeting with immunometabolic therapy. After systemic administration, magnetic actuation enhances immunobot retention and tumor penetration, while LPS‐functionalized FePt microrollers sustain M1‐like polarization through inflammatory and iron‐amplified oxidative signaling and trigger ...
Xirui Zeng, Yingfei Zhang, Chong Wu, Qijia Duan, Mengqi Fan, Kuan Wang, Shirong Cai, Immihan Ceren Yasa   +7 more
wiley   +1 more source

Impact of ER stress and the unfolded protein response on Fabry disease

EBioMedicine
Summary: Fabry disease (FD) is a lysosomal storage disorder caused by pathogenic missense and nonsense variants in the α-galactosidase A (GLA) gene, leading to absent or reduced enzyme activity.
Malte Lenders, Elisa Rudolph, Eva Brand
doaj   +1 more source

The Continuous Challenge of Diagnosing patients with Fabry disease in Argentina

Journal of Inborn Errors of Metabolism and Screening, 2015
The lysosomal storage disorder Fabry disease (FD) is caused by pathogenic mutations in the α-galactosidase A gene, localized in X chromosome. Deficient enzymatic activity of the product of this gene, the lysosomal hydrolase α-galactosidase A, leads to ...
Paula A Rozenfeld PhD, Romina Ceci Bioc, Norma Roa Lic, Isaac Kisinovsky MD   +3 more
doaj   +1 more source

Infantile nephropathic cystinosis with incomplete fanconi syndrome, hypothyroidism, hydro-uretero-nephrosis, and megacystis

Saudi Journal of Kidney Diseases and Transplantation, 2016
Cystinosis is an autosomal recessive lysosomal storage disorder characterized by the accumulation of the amino-acid cysteine in various organs and tissues. Infantile nephropathic cystinosis is the most severe form of the disorder.
Vaishali More, Preeti Shanbag
doaj   +1 more source

Neuron‐Derived Vesicular circMagi1 Restrains Vascular Repair After Ischemic Stroke by Promoting TRIM65‐Mediated ANXA2 Degradation

Advanced Science, EarlyView.
After ischemic stroke, injured neurons transfer circMagi1 to endothelial cells through extracellular vesicles. Within endothelial cells, circMagi1 binds ANXA2 and enhances its engagement with TRIM65, promoting ANXA2 ubiquitination and proteasomal degradation to constrain angiogenic repair.
Youkun Bi, Qun Liu, Baohua Zou, Wenyuan Wang, Xinlong Qiao, Lin Gao, Chaoxu Miao, Ningning Huang, Shuqing Tang, Rui Guo, Changqing Zeng, Ce Jia, Yuanchao Xue, Xuejun Guo, Jianjun Chen, Huiwen Wang, Guangju Ji   +16 more
wiley   +1 more source

Therapeutic applications of imino sugars in lysosomal storage disorders.

, 2016
The N-alkylated imino sugars have inhibitory activity against the first enzyme in the pathway for glucosylating sphingolipid in eukaryotic cells, ceramide-specific glucosyltransferase.

core   +1 more source

Developmental Environmental Chronotoxicity by Polystyrene Nanoplastics Exacerbates High‐Fat Diet‐Induced Pediatric MAFLD Through Circadian Disruption and Mitochondrial Dysfunction

Advanced Science, EarlyView.
Polystyrene nanoplastics exacerbate high‐fat diet‐induced metabolic dysfunction in an age‐dependent manner. Juvenile mice exhibit greater susceptibility, characterized by circadian disruption, impaired mitochondrial function, reduced energy metabolism, and enhanced hepatic lipid accumulation.
Peihao Xu, Shuting Huang, Leilei Zhu, Junyi Huang, Kewei Li, Guangming Li, Jiannong Wu, Mingyuan Zhou, Fangmei Zhou, Zhishan Ding, Yingzhi Shen   +10 more
wiley   +1 more source

Rapid Multisystem Deterioration After Enzyme Replacement Therapy Discontinuation in Fabry Disease: A Familial Case Series with Kidney Transplantation

Turkish Journal of Nephrology
Fabry disease (FD) is an X-linked lysosomal storage disorder with progressive kidney cardiac, and neurological involvement. Enzyme replacement therapy (ERT) remains the cornerstone of management; however, the clinical consequences of treatment ...
Erdem Baran
doaj   +1 more source

Restores Aβ Clearance by Overcoming PCSK9–LRP1 Dysregulation and TRIB3‐Mediated Autophagy Blockade in Alzheimer's Disease

Advanced Science, EarlyView.
PCSK9 upregulation in cerebrovascular endothelial cells and microglia destabilizes the Aβ transporter LRP1, while TRIB3‐associated autophagy blockade further limits intracellular Aβ degradation. A RAP‐modified tea‐polyphenol nanoparticle co‐delivers siPCSK9 and siTRIB3 to restore LRP1‐mediated Aβ efflux and autophagy‐dependent degradation, reducing Aβ ...
Jie Miao, Jing Wang, Yankun Li, Haipei Zhang, Yanli Zhang, Qingnian Li, Bo Xiao, Wenhu Zhou, Junhong Guo   +8 more
wiley   +1 more source

Modelling the neuropathology of lysosomal storage disorders through disease-specific human induced pluripotent stem cells [PDF]

, 2019
Mucopolysaccharidosis II (MPS II) is a lysosomal storage disorder (LSD), caused by iduronate 2-sulphatase (IDS) enzyme dysfunction. The neuropathology of the disease is not well understood, although the neural symptoms are currently incurable.
Nakanishi, H., Laszlo, L., Lo Giudice, Maria, László, L, Pijnappel, WWM Pim, Varga, Norbert, Kobolák, J, Kitiyanant, N, Hyttel, Poul, Bock, István, Jezsó, B, Hyttel, P., Zhou, S, Pijnappel, Pim, Jerso, B., Zhou, SL, Téglási, A, Varga, Eszter, Jerso, B, Pijnappel, WWM P, Dinnyés, A, Pijnappel, W., Dinnyés, András, Varga, N., Freude, K., Freude, K, Hyttel, P, Westerveld, M. (Michael), Phanthong, P., Nakanishi, H, Molnar, K., Dinnyes, A., Nakanishi, Hideyuki, Lo Giudice, M, Bock, I., Bock, I, Kobolák, Julianna, Hoogeveen-Westerveld, M, Hoogeveen - Westerveld, Marianne, Kitiyanant, Narisorn, Varga, E, Freude, Kristine, Phanthong, Phetcharat, Kitiyanant, N., Varga, N, Varga, E. (Endre), Teglasi, A., Jezsó, Bálint, Molnár, Kinga, Molnár, K, László, Lajos, Kobolak, J., Zhou, Shuling, Phanthong, P, Lo Giudice, M., Hoogeveen-Westerveld, Marianne, Zhou, S.L., Téglási, Annamária   +57 more
core   +1 more source