Results 81 to 90 of about 6,268,347 (286)
THE LYSOSOMAL STORAGE DISEASE GM2 GANGLIOSIDOSIS IN CAPTIVE BANDED MONGOOSE SIBLINGS (MUNGOS MUNGO).
, 2018 This study reports the occurrence of the lysosomal storage disease GM2 gangliosidosis (Sandhoff disease) in two 11-mo-old captive-bred, male and female mongoose siblings ( Mungos mungo). The clinical signs and the pathological findings reported here were
Wenker, Christian +17 more
core +1 more source
Advanced Materials, EarlyView.Lignin‐mimicking protein methylation can enhance the efficacy and mitigate the toxicity of the classic FOLFOX chemotherapy regimen. ABSTRACT FOLFOX has served as the standard chemotherapy regimen for advanced stages, specifically in the treatment of pancreatic, colorectal, and bladder cancers.
Shiyao Song +14 more
wiley +1 more source
A European Consortium for Lysosomal Storage Diseases
, 2008 Lysosomes are membrane-enclosed compartments, filled with hydrolytic enzymes that are used for the degradation of macromolecules. Proteins and other substrates are delivered to the lysosomes by various pathways including endocytosis, and autophagy, a ...
ANDRIA, GENEROSO
core
Advanced Materials, EarlyView.An engineered Trojan Mycobacterium, developed via a “peeling‐off and masking‐up” strategy, functions as a powerful splenic myelopoiesis converter. This spleen‐targeted nanomedicine induces trained immunity to reprogram immunosuppressive myeloid reservoirs into antitumoral effectors.
Jin‐Ho Choi +5 more
wiley +1 more source
Advanced Science, EarlyView.The role of TNFα in the process of renal allograft interstitial fibrosis is complex and multifaceted. As it can promote renal allograft interstitial fibrosis by inducing mitochondrial dysfunction and EndMT, and also mediating compensatory mitophagy through the NEDD4–HIF‐1α–BNIP3 pathway, which clears damaged mitochondria and inhibits EndMT, thereby ...
Dengyuan Feng +15 more
wiley +1 more source
Mr BMT Achieves Systemic Macrophage Replacement With Preservation of Tissue Homeostasis
Advanced Science, EarlyView.Microglia replacement by bone marrow transplantation (Mr BMT) enables systemic replacement of tissue‐resident macrophages. Despite persistent macrophage and tissue remodeling across multiple organs, core biological functions and innate immune responses remain preserved, supporting long‐term maintenance of organismal homeostasis and the therapeutic ...
Yufei Xu +17 more
wiley +1 more source
Innate Immunocompetent hiPSC‐Derived Neurospheroids Capture Early CNS Responses to rAAV
Advanced Science, EarlyView.Knowledge of human CNS immune responses to AAV‐based gene therapies remains limited due to the lack of immune‐competent human models. Here, a hiPSC‐derived 3D neuroimmune platform integrating neurospheroids and microglia is established using stirred‐tank bioreactors.
Catarina M. Gomes +14 more
wiley +1 more source
Charting Endocrine Progenitors Across Species and Organs
Advanced Science, EarlyView.Endocrine progenitors give rise to the hormone‐producing cells of the pancreas and intestine. Using single‐cell multiomics and proteomics, this study compares these progenitors across species, systems, and organs, mapping the conserved and species‐specific gene regulatory networks that guide their formation.
Changying Jing +21 more
wiley +1 more source
Golgi defect as a major contributor to lysosomal dysfunction
Frontiers in Cell and Developmental BiologyThe Golgi apparatus plays a crucial role in lysosome biogenesis and the delivery of lysosomal enzymes, essential for maintaining cellular homeostasis and ensuring cell survival. Deficiencies in Golgi structure and function can profoundly impact lysosomal
Sarah R. Akaaboune, Yanzhuang Wang
doaj +1 more source
International Journal of Neonatal Screening, 2019 The increasing availability of treatments and the importance of early intervention have stimulated interest in newborn screening for lysosomal storage diseases.
Alberto B. Burlina +7 more
doaj +1 more source