Results 71 to 80 of about 8,108 (242)

G3BP1 Succinylation at K413 is Critical for Cardiac Function by Modulating PI3K‐AKT‐mTOR Signal Axis

Advanced Science, EarlyView.
Schematic illustrating the impact of G3BP1 succinylation at K413 on cardiac function. In the healthy human heart, G3BP1 succinylation maintains homeostatic mTOR signaling. In patients with dilated cardiomyopathy (DCM) and heart failure (HF), G3BP1 de‐succinylation induces RagA expression and disrupts the binding of the TSC1/2 complex, leading to the ...
Yuan Zhang, Cancan Yao, Yan Chen, Ke Cai, Zhouping Lu, Boxuan Wu, Kun Yu, Yan Shi, Jianyuan Zhao, Xiangyu Zhou   +9 more
wiley   +1 more source

Chronic intestinal pseudo-obstruction. Did you search for lysosomal storage diseases?

Molecular Genetics and Metabolism Reports, 2017
Chronic intestinal pseudo-obstruction results in clinical manifestations that resemble intestinal obstruction but in the absence of any physical obstructive process.
J. Politei, C. Durand, A.B. Schenone, A. Torres, J. Mukdsi, B.L. Thurberg   +5 more
doaj   +1 more source

Tumor‐Derived Alpha‐1 Antitrypsin Promotes Liver Metastasis of Colorectal Cancer Through the Neutrophil Extracellular Traps–CCDC25 Pathway

Advanced Science, EarlyView.
ABSTRACT Liver metastasis is a leading cause of mortality in colorectal cancer (CRC), where the inflammatory tumor microenvironment, specifically neutrophil infiltration, significantly promotes metastatic colonization. This study reveals a pro‐metastatic role for alpha‐1 antitrypsin (A1AT) in CRC liver metastasis via a dual mechanism involving ...
Qian Fei, Chunning Li, Lei Zhan, Xiaoyan Li, Yan Hui, Yue Jin, Jing Zhang, Lu Yang, Xin Sun, Xiaoxi Li, Qian Dong, Jingdong Zhang   +11 more
wiley   +1 more source

Tau accumulation in degradative organelles is associated to lysosomal stress

Scientific Reports, 2023
Neurodegenerative disorders are characterized by the brain deposition of insoluble amyloidogenic proteins, such as α-synuclein or Tau, and the concomitant deterioration of cell functions such as the autophagy-lysosomal pathway (ALP).
Ester Piovesana, Claudia Magrin, Matteo Ciccaldo, Martina Sola, Manolo Bellotto, Maurizio Molinari, Stéphanie Papin, Paolo Paganetti   +7 more
doaj   +1 more source

Lysosomal Storage Diseases

Journal of Inborn Errors of Metabolism and Screening, 2014
Lysosomal storage diseases are a group of inherited and acquired disorders. They are characterized by interruption of recycling of cellular and extracellular molecules. Clinically, they are presented as developmental and neurological symptoms similar to other inherited and acquired disorders.
Alroy, Joseph, Lyons, Jeremiah A.
openaire   +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 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  

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

Glycolipid lysosomal storage disease in a Morgan foal [PDF]

, 2020
Lysosomal storage diseases are a group of inherited and acquired disorders affecting mammals and birds in which specific substrates accumulate in lysosomes as a result of deficient activity of lysosomal hydrolases.
Lozza, Facundo Andrés, Bellamy, P. D., Barr, B. C., Loretti, A. P., Gimeno, Eduardo Juan, Nordhausen, R. W.   +5 more
core  

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