Results 101 to 110 of about 58,222 (243)

Multiplexing Iduronate-2-Sulphatase (MPS-II) into a 7-Plex Lysosomal Storage Disorder MS/MS Assay Using Cold-Induced Phase Separation. [PDF]

Int J Neonatal Screen, 2023
Courtney E, Pickens CA, Cuthbert C, Petritis K.   +3 more
europepmc   +1 more source

Evaluation of the lysosome-associated membrane protein LAMP-2 as a marker for lysosomal storage disorders [PDF]

, 1998
Chi T. Hua, John J. Hopwood, Sven R. Carlsson, R. J. Harris, Peter J. Meikle   +4 more
openalex   +1 more source

Stimuli‐Responsive Supramolecular Biomaterials for Cancer Theranostics

Advanced Science, EarlyView.
The ultimate goal of cancer theranostics is to get imaging agents and therapeutic cargo to tumor sites when and where they are required. “Smart” systems should be developed. This review discusses the characteristics of physiological stimuli, types and action modes of external stimuli, construction approaches and working principles, as well as ...
Wenting Hu, Binglin Ye, Guocan Yu, Feihe Huang, Zhengwei Mao   +4 more
wiley   +1 more source

Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease. [PDF]

Cells, 2023
El-Hage N, Haney MJ, Zhao Y, Rodriguez M, Wu Z, Liu M, Swain CJ, Yuan H, Batrakova EV.   +8 more
europepmc   +1 more source

Is Parkinson's disease a lysosomal disorder?

Brain : a journal of neurology, 2018
Common forms of Parkinson's disease have long been described as idiopathic, with no single penetrant genetic factor capable of influencing disease aetiology.
A. Klein, Joseph R. Mazzulli
semanticscholar   +1 more source

Acetylation Regulates ACSL4 Degradation Through Chaperone‐Mediated Autophagy to Alleviate Intervertebral Disc Degeneration

Advanced Science, EarlyView.
Intervertebral disc degeneration is triggered by ACSL4 accumulation‐mediated ferroptosis of nucleus pulposus cells due to CMA dysfunction. KAT2B promotes ACSL4 degradation via CMA through acetylation. AAV‐mediated LAMP2A delivery or engineered exosomes rescue nucleus pulposus cell senescence and disc degeneration.
Zhouwei Wu, Zhichen Jiang, Chenglong Hong, Shu Yang, Shuqing Jin, Chenyu Wu, Kaijie Guo, Jiang Liu, Shaobo Xu, Chenggui Wang, Xiangyang Wang   +10 more
wiley   +1 more source

Control of proteinuria with increased doses of agalsidase alfa in a patient with Fabry disease with atypical genotype–phenotype expression

Nefrología, 2015
Fabry disease is a rare X-linked lysosomal storage disorder of glycosphingolipids, caused by the partial or complete deficiency of the lysosomal enzyme alpha-galactosidase A (a-Gal A).
Christos Paliouras, Georgios Aperis, Foteini Lamprianou, Giorgos Ntetskas, Konstantinos Roufas, Polichronis Alivanis   +5 more
doaj   +1 more source

Lysosomal storage disorders: Present and future [PDF]

Indian Pediatrics, 2015
ysosomal storage disorders (LSDs) is a group of more than 50 single gene disorders caused by deficiencies of any of the glycoprotein acid hydrolase enzymes. Lysosomes are intracellular organelles serving important functions of breakdown of large molecules and helping in their disposal.
openaire   +2 more sources

Neuroprotective Effects of Time‐Restricted Feeding Combined With Different Protein Sources in MPTP‐Induced Parkinson's Disease Mice Model and Its Modulatory Impact on Gut Microbiota Metabolism

Advanced Science, EarlyView.
Time‐restricted feeding (TRF) exerts protein‐dependent neuroprotective effects in an MPTP‐induced Parkinson's disease model. In casein‐fed mice, TRF improves gut barrier integrity and reduces neuroinflammation, possibly via modulation of Allobaculum and BCAAs.
Ting Li, Jian Wu, Sheng‐Yang Zhou, Ming‐An Li, Li‐Ping Zhao, Ao Wang, Yi‐Zhi Song, Wen‐Yan Huang, Lu‐Lu Tan, Chen‐Meng Qiao, Wei‐Jiang Zhao, Chun Cui, Yan‐Qin Shen   +12 more
wiley   +1 more source

A perspective on research, diagnosis, and management of lysosomal storage disorders in Colombia [PDF]

, 2020
María Alejandra Puentes-Tellez, Paula Andrea Lerma-Barbosa, Rafael Guillermo Garzón-Jaramillo, Diego A. Súarez, Ángela J. Espejo, Johana Guevara, Olga Yaneth Echeverri-Peña, Daniela Solano-Galarza, Alfredo Uribe, Carlos Javier Alméciga-Díaz   +9 more
openalex   +1 more source