Results 111 to 120 of about 84,505 (291)

Purification of the lysosomal sialic acid transporter. Functional characteristics of a monocarboxylate transporter [PDF]

, 1998
Sialic acid and glucuronic acid are monocarboxylated monosaccharides, which are normally present in sugar side chains of glycoproteins, glycolipids, and glycosaminoglycans.
Beerens, C.E.M.T. (Cecile), Havelaar, A.C. (Adrie), Mancini, G.M.S. (Grazia), Souren, R.M., Verheijen, F.W. (Frans)   +4 more
core   +4 more sources

Liquid Metal Nanotransformers for Drug‐Resistant Pan‐Cancer Therapy in Patient‐Derived Organoids

Advanced Science, EarlyView.
Pan‐cancer therapies are severely limited in drug‐resistance patients due to genetic mutations and other factors, resulting in poor therapeutic outcomes and constrained clinical benefit. Liquid metal nanotransformers, a new class of shape‐transformable nanomaterials capable of dramatic morphological changes, offer a promising physical strategy to ...
Xiaojie Yuan, Xuelin Wang, Zhongyao Chen, Shuo Wang, Weining Gao, Ziyi Chen, Runyang Li, Cheng Hao, Lei Zhang, Qiongqiong Zhang, Ke Zhang, Mei Yu, Qian Liu, Jingxuan Wang, Huiping Li, Simpkins Fiona, Jing Liu, Qiang Liu, Peng Liu, Yawei Hu   +19 more
wiley   +1 more source

A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

Disease Models & Mechanisms, 2016
Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates.
Heike Wolf, Markus Damme, Stijn Stroobants, Rudi D'Hooge, Hans Christian Beck, Irm Hermans-Borgmeyer, Renate Lüllmann-Rauch, Thomas Dierks, Torben Lübke   +8 more
doaj   +1 more source

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice [PDF]

, 2015
OBJECTIVE: The lysosomal storage disease alpha‐mannosidosis is caused by the deficiency of the lysosomal acid hydrolase alpha‐mannosidase (LAMAN) leading to lysosomal accumulation of neutral mannose‐linked oligosaccharides throughout the body, including ...
Andersson, Claes, Beck, Hans Christian, Blanz, Judith, Damme, Markus, D’Hooge, Rudi, Ericsson, Annika, Fogh, Jens, Lüdemann, Meike, Lüllmann-Rauch, Renate, Rothaug, Michelle, Saftig, Paul, Stroobants, Stijn   +11 more
core   +2 more sources

Cuproptosis and Mitophagy Mediated by the THUMPD1/IGF2R‐Dependent Suppression of AKT and Activation of AMPK Signaling Suppress Lung Adenocarcinoma Progression

Advanced Science, EarlyView.
THUMPD1 drives a tumor‐suppressive signaling cascade in lung adenocarcinoma by promoting IGF2R expression. IGF2R associates with PPP2R1A to suppress AKT and activate AMPK, leading to SLC31A1 upregulation and copper accumulation. Elevated copper disrupts mitochondrial metabolism and induces excessive mitophagy, thereby restraining tumor growth and ...
Kai Wu, Bo Qin, Zhuoyu Gu, Weizheng Ding, Xiaoming Chen, Kaishang Zhang, Yujin Qiao, Shuang Yuan, Song Zhao, Xiangnan Li, Peng Zhang   +10 more
wiley   +1 more source

Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H. [PDF]

, 2016
Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs).
Dickson, Patricia I, Lund, Troy C, Miller, Weston P, Orchard, Paul J, Pasquali, Marzia, Polgreen, Lynda E, Raymond, Gerald V   +6 more
core   +1 more source

Dose‐Dependent Reprogramming of Chromatin Accessibility by SOX4 Drives the Transcriptional Response to Iron Overload

Advanced Science, EarlyView.
This study demonstrates that iron overload triggers widespread chromatin compaction and transcriptional repression in human granulosa cells, recapitulating features of endometriosis. The epigenetic reprogramming is orchestrated by a TFEB‐SOX4‐SWI/SNF axis, with SOX4 acting as a central, dosage‐sensitive regulator.
Feifei Li, Yaoqiu Wu, Guangyu Yang, Jingyi Lai, Xiaoyue Sun, Liyan Wang, Xiaoli Li, Jing Zhang, Qingxue Zhang, Hui Chen, Haiyan Lin, Bingxiang Xu, Junfeng Zhang, Hailong Wang, Anming Meng, Chunwei Cao   +15 more
wiley   +1 more source

Biochemical and clinical response after umbilical cord blood transplant in a boy with early childhood-onset beta-mannosidosis. [PDF]

, 2019
BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development ...
Eisengart, Julie B., Lund, Troy C., Miller, Weston P., Orchard, Paul J., Patterson, Marc C., Pollard, Laura, Renaud, Deborah L., Simmons, Katrina, Wenger, David A.   +8 more
core   +1 more source

BZW1 Drives Immune Evasion in Lung Adenocarcinoma via Ferroptosis Suppression

Advanced Science, EarlyView.
BZW1 attenuates ferroptosis by competitively binding NCOA4 and suppress ferrtinophagy‐mediated iron release. The depletion of BZW1 triggers lipid peroxidation through iron homeostasis. Extracellularly, BZW1 attenuates immunogenic cell death and reinvigorates cytotoxic T‐cell responses.
Linyao Zhao, Yue Peng, Qing Liang, Shi Liu, Yang Li, Lei Ma, Menghan Hu, Sujuan Zheng, Zhihua Liu, Shugeng Gao   +9 more
wiley   +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