Results 121 to 130 of about 42,118 (292)

The Association Between Lysosomal Storage Disorder Genes and Parkinson's Disease: A Large Cohort Study in Chinese Mainland Population. [PDF]

Front Aging Neurosci, 2021
Zhao YW, Pan HX, Liu Z, Wang Y, Zeng Q, Fang ZH, Luo TF, Xu K, Wang Z, Zhou X, He R, Li B, Zhao G, Xu Q, Sun QY, Yan XX, Tan JQ, Li JC, Guo JF, Tang BS.   +19 more
europepmc   +1 more source

Loss of VMP1 Impairs Tight Junction Recycling and Aggravates Intestinal Barrier Dysfunction in Inflammatory Bowel Disease

Advanced Science, EarlyView.
This study identifies vacuole membrane protein 1 (VMP1) as a critical regulator of intestinal epithelial barrier homeostasis. VMP1 facilitates the recruitment of CORO1C to late endosomes, supporting Retromer‐mediated recycling of the tight junction protein Occludin.
Jiawei Zhao, Jianjun Zou, Chen Zhou, Yingui Wang, Yiman Liu, Yanqing Zhou, Yuxiang Wang, Xinyu Zhang, Huishu Yang, Hongjie Yin, Dongsheng Bai, Yue Zhao, Na Lu   +12 more
wiley   +1 more source

Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic. [PDF]

Cell, 2021
Logan T, Simon MJ, Rana A, Cherf GM, Srivastava A, Davis SS, Low RLY, Chiu CL, Fang M, Huang F, Bhalla A, Llapashtica C, Prorok R, Pizzo ME, Calvert MEK, Sun EW, Hsiao-Nakamoto J, Rajendra Y, Lexa KW, Srivastava DB, van Lengerich B, Wang J, Robles-Colmenares Y, Kim DJ, Duque J, Lenser M, Earr TK, Nguyen H, Chau R, Tsogtbaatar B, Ravi R, Skuja LL, Solanoy H, Rosen HJ, Boeve BF, Boxer AL, Heuer HW, Dennis MS, Kariolis MS, Monroe KM, Przybyla L, Sanchez PE, Meisner R, Diaz D, Henne KR, Watts RJ, Henry AG, Gunasekaran K, Astarita G, Suh JH, Lewcock JW, DeVos SL, Di Paolo G.   +52 more
europepmc   +1 more source

ALKBH3 m1A Demethylase Deficiency Reduces Alzheimer's Amyloid‐β Pathology

Advanced Science, EarlyView.
This study identifies that ALKBH3‐driven m1A demethylation orchestrates Alzheimer's disease progression by disrupting mitochondrial and synaptic homeostasis. This epitranscriptomic mechanism suppresses PINK1‐mediated mitophagy via m1A erasure, leading to mitochondrial dysfunction, oxidative stress, elevated Aβ production, and impaired microglial ...
Yueyang Li, Sifei Yu, Kaidong Lu, Yujie Zhang, Mingjie Dong, Yan Peng, Liang Xue, Waleed Alam, Yuxuan Shui, Yi Zhou, Wuyunhan Ma, Meng Bao, Peiming Li, Peiyi Luo, Tiezhan Lu, Jiajia Li, Kang Zhang, Yuying Wang, Shuchen Yang, Nuoya Yin, Francesco Faiola, Zilong Gao, Jingfeng Zhou, Fei Zhao, Yali He, Magdalena J. Koziol   +25 more
wiley   +1 more source

Deficiency in NPC2 results in disruption of mitochondria-late endosome/lysosomes contact sites and endo-lysosomal lipid dyshomeostasis

Scientific Reports
Dysfunction of the endo-lysosomal intracellular Cholesterol transporter 2 protein (NPC2) leads to the onset of Niemann–Pick Disease Type C (NPC), a lysosomal storage disorder.
Raffaele Pastore, Lihang Yao, Nathan Hatcher, Martin Helley, Janet Brownlees, Radha Desai   +5 more
doaj   +1 more source

Neuronal genetic rescue normalizes brain network dynamics in a lysosomal storage disorder despite persistent storage accumulation. [PDF]

Mol Ther, 2022
Ahrens-Nicklas RC, Tecedor L, Hall AF, Kane O, Chung RJ, Lysenko E, Marsh ED, Stein CS, Davidson BL.   +8 more
europepmc   +1 more source

NIR‐II Imaging‐Guided Photothermal Activation of a TRPV4‐Targeted Nanoplatform Delivering Cycloastragenol to Promote Microglia Reprogramming and α‐Synuclein Clearance in Parkinson's Disease

Advanced Science, EarlyView.
ABSTRACT Current therapies for Parkinson's disease (PD) fail to concurrently address α‐synuclein (α‐syn) aggregation and microglia‐mediated neuroinflammation. Herein, we engineer a near‐infrared‐II (NIR‐II) phototheranostic nanoplatform, CAG/FD1080@MM‐aTRPV4, for synergistic regulation of microglial function and real‐time monitoring of PD pathology. We
Hsuan Lo, Linjuan Feng, Shiying Li, Lik Hang Hugo Tse, Xuehan Wang, Xingyang Zhao, Shaoheng Ma, Xin Li, Yanjuan Gu, Wing‐Tak Wong   +9 more
wiley   +1 more source

A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder. [PDF]

J Inherit Metab Dis, 2022
Hassall S, Smith DM, Rust S, Wittkowski A.   +3 more
europepmc   +1 more source

Tumor‐Induced Splenic Remodeling: Mechanisms of Systemic Immunosuppression and Emerging Therapeutic Opportunities

Advanced Science, EarlyView.
ABSTRACT Despite the transformative impact of cancer immunotherapies such as immune checkpoint blockade, durable clinical responses remain limited. Increasing evidence indicates that antitumor immunity is governed not only by the tumor microenvironment, but also by systemic immune regulation mediated by peripheral immune organs. Among these, the spleen
Yuehua Liu, Xiaoqian Nie, Xiaofei Gao
wiley   +1 more source

Nanozymes for Liver Disease Therapy: Advances in Catalytic Activity, Targeting Strategies, and Clinical Translation

Advanced Science, EarlyView.
Nanozymes, as enzyme‐mimicking nanomaterials, exhibit unique catalytic properties for the treatment of liver diseases. By regulating redox homeostasis, modulating immune responses, and enabling targeted delivery, nanozymes overcome the limitations of natural enzymes.
Xiandi Meng, Ge Zhu, Siyu Sun, Wenbo Yao, Yuning Zhang, Yong‐Guang Yang, Tianmeng Sun   +6 more
wiley   +1 more source