Results 171 to 180 of about 3,952,882 (246)

Disrupted TFEB/GDNF–cAMP/ATP Coupling Underlies Astrocytic Dysfunction and Depression in LRRK2 G2019S Parkinson's Mice

Advanced Science, EarlyView.
ABSTRACT The LRRK2 G2019S mutation, a Parkinson's disease–linked variant, has been associated with depression‐like phenotypes, but mechanisms remain unclear. We chart age‐dependent behavioral changes and astrocyte reactivity in G2019S mice and define a pathway connecting LRRK2 to TFEB/GDNF signaling, cellular energetics, and inflammation.
Longping Yao, Maryam Hatami, Sumeyye Koc, Qing Jiang, Thomas Skutella, Yuanfeng Zhang   +5 more
wiley   +1 more source

Diagnosis and follow-up of the first case of Gaucher disease under enzyme replacement therapy in Senegal. [PDF]

Ghana Med J
Keita M, Faye BF, Senghor AB, Diallo AB, Bousso ES, Touré SA, Seck M, Diop S.   +7 more
europepmc   +1 more source

Targeted Restoration of the Microbial Tryptophan Oxidative Pathway Ameliorates Autism‐Like Behavioral and Synaptic Deficits

Advanced Science, EarlyView.
ABSTRACT Autism Spectrum Disorder (ASD) is associated with gut microbiota dysbiosis, yet the contribution of specific microbial metabolic pathways remains unclear. Reanalysis of a public ASD cohort revealed reduced functional potential in microbial tryptophan oxidative metabolism.
Rui Guo, Caian He, Xiao Xiao, Jingmeng Li, Zilong Zhang, Ying He, Chuanchuan Wang, Jiale Zhao, Jun Gong, Jiarui Liang, Tian Yuan, Haiting Sun, Xuebo Liu, Chao Gao, Zhigang Liu   +14 more
wiley   +1 more source

Stability of alglucosidase alfa in 0.9% sodium chloride for enzyme replacement therapy in patients with Pompe disease: insights from enzyme activity and cellular uptake measurements. [PDF]

Eur J Hosp Pharm
Barzel I, Brugma JC, Jacobs EH, Hoogeveen-Westerveld M, van der Kuy PHM, Preijers T.   +5 more
europepmc   +1 more source

Co‐Delivery of Sustained Release Chondroitinase ABC‐37 With Human iPSC‐Derived Neural Progenitors Promotes Transplant Survival and Functional Recovery in a Rodent Model of Stroke

Advanced Science, EarlyView.
A regenerative strategy for stroke combines human stem cell–derived neural progenitor cells with sustained release of a matrix‐modifying, thermostable chondroitinase ABC‐37 enzyme. In a rat model of stroke, co‐delivery enhanced transplanted cell survival and neuronal differentiation, degraded inhibitory extracellular matrix components, and improved ...
Nitzan Letko Khait, David Xinzheyang Li, Keyi Guo, Hong Cui, Quinton Sirianni, Dhana Abdo, Cindi M. Morshead, Molly S. Shoichet   +7 more
wiley   +1 more source

Enzyme replacement therapy during pregnancy and breastfeeding in late-onset Pompe disease. [PDF]

Int Breastfeed J
Bachmann M, Kasper D, Scholl-Bürgi S, Margreitter J, Höller A, Löscher W, Karall D, Zöggeler T.   +7 more
europepmc   +1 more source

Microglial Deubiquitinase OTUD7B Stabilizes STAT3 to Promote Neuroinflammation and Cognitive Decline in Alzheimer's Disease

Advanced Science, EarlyView.
. ABSTRACT Neuroinflammation driven by microglial activation is a defining feature of Alzheimer's disease (AD), yet the molecular mechanisms sustaining this proinflammatory state remain unclear. Here, we identify the deubiquitinase OTUD7B as a critical regulator of microglial activation and AD pathology.
Luyao Li, Hao Tang, Qin Yu, Yuyang Zhang, Binglu Shi, Ying Kong, Leiyu Xu, Jingjing Shao, Chenghong Hu, Lingyu She, Zhe Wang, Haiyi Chen, Aleksandr V. Samorodov, Gang Cao, Xia Zhao, Yi Wang   +15 more
wiley   +1 more source

Study Design Considerations for the Evaluation of Pancreatic Enzyme Replacement Therapy (PERT) Products in People with Cystic Fibrosis: An Update. [PDF]

Clin Ther
Konstan MW, Ramsey ML, Sathe M, Freeman AJ, Freedman SD, Schwarzenberg SJ, O'Rourke C, Onchiri FM, Setiawan L, Pilewski JM, Mayer-Hamblett N.   +10 more
europepmc   +1 more source

Lactiplantibacillus Plantarum GUANKE Alleviates Allergic Asthma by Restricting Monocyte‐to‐DC Differentiation Via Microbiota‐Associated IAA

Advanced Science, EarlyView.
Lactiplantibacillus plantarum GUANKE remodels the indigenous gut microbiota and is associated with microbiota‐dependent restoration of circulating IAA. IAA treatment attenuates JAK3‐STAT3 and Jag1‐Notch4‐Hey1 signaling and reduces CX3CR1+Ly6C− monocytic precursor generation and pulmonary CD11b+ DC accumulation, together with reduced Th2 inflammation ...
Yujia He, Jielan Mi, Zhiping Zhao, Xiao Zhang, Zhihan Yang, Kun Yue, Yuanming Huang, Liqiong Song, Xiaoting Wen, Jianguo Xu, Zhihong Ren   +10 more
wiley   +1 more source

Case Report: Is it COPD? It is Fabry disease: a case in which bronchodilators were briefly used but not continued, prioritizing enzyme replacement therapy. [PDF]

Front Pharmacol
Li Y, Zhu H, Zhan Z, Li G, Huang S, Xiao C, Li Y, Zheng C, Huang F.   +8 more
europepmc   +1 more source