Results 61 to 70 of about 40,385 (227)

Lysosomal Ca(2+) homeostasis: role in pathogenesis of lysosomal storage diseases.

, 2011
Disrupted cellular Ca(2+) signaling is believed to play a role in a number of human diseases including lysosomal storage diseases (LSD). LSDs are a group of ∼50 diseases caused predominantly by mutations in lysosomal proteins that result in accumulation ...
Lloyd-Evans, E, Platt, Frances, Platt, Frances M., Emyr Lloyd-Evans, Platt, FM, Frances M. Platt, Lloyd-Evans, Emyr   +6 more
core   +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

Lysosomal exocytosis and lipid storage disorders

Journal of Lipid Research, 2014
Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a
Mohammad Ali Samie, Haoxing Xu
doaj   +1 more source

Pre-clinical Mouse Models of Neurodegenerative Lysosomal Storage Diseases

Frontiers in Molecular Biosciences, 2020
There are over 50 lysosomal hydrolase deficiencies, many of which cause neurodegeneration, cognitive decline and death. In recent years, a number of broad innovative therapies have been proposed and investigated for lysosomal storage diseases (LSDs ...
Jacob M. Favret, Nadav I. Weinstock, M. Laura Feltri, Daesung Shin   +3 more
doaj   +1 more source

Rheumatologic Manifestations of Lysosomal Storage Diseases

, 2012
How to Cite this Article: Shiari R, VAdood Javadi P. Rheumatologic Manifestations of Lysosomal Storage Diseases. Iran J Child Neurol Autumn 2012; 6:4 (suppl. 1): 20. Pls see PDF.
SHIARI, Reza, JAVADI PARVANEH, Vadood
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

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

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  

Senolytic Therapy as a Preventive Strategy for Spine Degeneration and Pain

Advanced Science, EarlyView.
Cellular senescence promotes inflammation, tissue degeneration, and chronic back pain. In sparc‐null mice, early oral administration of the senolytic agents o‐vanillin and RG‐7112 reduced senescent cell burden and pro‐inflammatory SASP signaling across intervertebral discs, endplates, vertebral bone, and spinal cord.
Saber Ghazizadeh, Hosni Cherif, Matthew Mannarino, Juiena Sagir, Magali Millecamps, Jean A. Ouellet, Laura S. Stone, Lisbet Haglund   +7 more
wiley   +1 more source

Pompe Disease: New Developments in an Old Lysosomal Storage Disorder

Biomolecules, 2020
Pompe disease, also known as glycogen storage disease type II, is caused by the lack or deficiency of a single enzyme, lysosomal acid alpha-glucosidase, leading to severe cardiac and skeletal muscle myopathy due to progressive accumulation of glycogen ...
Naresh K. Meena, Nina Raben
doaj   +1 more source