Results 91 to 100 of about 154,911 (267)

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, Miaozhan Zou, Yunshang Bai, Yang He, Zhihao Jin, Baozhi Yang, Jinjin Du, Pei Ouyang, Yuanyuan Cai, Yuheng Zhang, Bingying Du, Chengcheng Ge, Jian‐an Gong, Xiaoyu Li, Yuanyi Zheng, Jing Ding, Bo Peng, Yanxia Rao   +17 more
wiley   +1 more source

Mechanism of Secondary Ganglioside and Lipid Accumulation in Lysosomal Disease. [PDF]

Int J Mol Sci, 2020
Breiden B, Sandhoff K.
europepmc   +1 more source

BAP31 Drives Cartilage Calcification through Disruption of Autophagosome–Lysosome Fusion in Osteoarthritis

Advanced Science, EarlyView.
Mechanical stress activates BAP31 in chondrocytes. BAP31 competes with ATG14 for binding to STX17, disrupting the STX17–ATG14 complex required for autophagosome–lysosome fusion. The resultant autophagic flux blockade drives the generation of autophagy‐derived exosomes, which mediate pathological cartilage calcification in OA. Chondrocyte‐targeted BAP31
Zhi‐hua Xu, Ruo‐xin Wang, Feng He, Qian Liu, Fu‐yin Li, Min Hui, Yu‐qian Shi, Yu Jiang, Qing‐hua Li, Jun‐wen Jing, Shi‐bin Yu, Li‐tian Ma, Qian‐nan Niu, Mian Zhang   +13 more
wiley   +1 more source

Lysosomal homeostasis at the crossroads of neurodegeneration

The Journal of Clinical Investigation
Lysosomes function as metabolic control centers that integrate degradation, nutrient sensing, and stress signaling. In neurons, which must maintain proteostasis and energetic balance throughout life, lysosomal homeostasis determines cellular resilience ...
Stefano De Tito, Sharon A. Tooze
doaj   +1 more source

Broken Balance: Emerging Cross-Talk Between Proteostasis and Lipostasis in Neurodegenerative Diseases

Cells
Neurodegenerative diseases, including Alzheimer’s disease and Parkinson’s disease, are characterized by progressive neuronal loss, leading to cognitive and motor impairments.
Jessica Tittelmeier, Carmen Nussbaum-Krammer   +1 more
doaj   +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, Gabriela Silva, Mafalda Aleixo, Marta Gomes, Daniel Simão, Stephan J. Holtkamp, Diana D. Lobo, Pradeep Harish, Rosalind Jenkins, Lekh N. Dahal, Rui J. Nobre, Luís de Pereira de Almeida, Mark Trautwein, Paula M. Alves, Catarina Brito   +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, Michael Sterr, Özüm Sehnaz Caliskan, Lama Saber, Nicole Katarina Rogers, Samuel Ogden, Kei Kozawa, Christos Karampelias, Jessica Jaki, Aimée Bastidas‐Ponce, Falk Janos Farkas, Oliver Czarnecki, Osvaldo Rodrigues Pereira, Perla Cota, Valerie Vandenbempt, Diego Balboa, Anika Böttcher, Natalie Krahmer, Mohammad Lotfollahi, Julie B. Sneddon, Heiko Lickert, Mostafa Bakhti   +21 more
wiley   +1 more source

Effect of disease progression on CSF-directed AAV gene therapy in a large brain animal model of lysosomal storage disease

Molecular Therapy: Methods & Clinical Development
The lysosomal storage disease alpha-mannosidosis (AMD) is caused by a genetic deficiency of lysosomal alpha-mannosidase, leading to the widespread presence of storage lesions in the brain and other tissues.
Jacqueline E. Hunter, Caitlyn M. Molony, Wojciech K. Panek, Charles H. Vite, Sanjeev Chawla, Harish Poptani, John H. Wolfe   +6 more
doaj   +1 more source

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

ATP6V0A1 protects dopaminergic neurons via the autophagy–lysosomal pathway in Parkinson’s disease

Neural Regeneration Research
Parkinson’s disease is the second most common neurodegenerative disorder. ATPase H+ transporting V0 subunit A1 (ATP6V0A1) is a component of vacuolar H+-ATPase (V-ATPase), an ATP-dependent proton pump.
Yuwan Lin, Zixin Tan, Wenfeng Ye, Weimin Li, Hao Chen, Yuping Lin, Miaomiao Zhou, Hanqun Liu, Qin Liu, Zhiling Zhang, Weixin Kong, Zongtang Xu, Hao Lin, Mingshu Mo, Wenyuan Guo, Keye Lin, Jiaxin Tang, Yueying Zheng, Wenlong Zhang, Pingyi Xu, Xiang Chen   +20 more
doaj   +1 more source