Results 11 to 20 of about 87,336 (259)

Lysosomal Storage Diseases: Heterogeneous Group of Disorders [PDF]

BioImpacts, 2013
The name of lysosomal storage diseases stems from the fact that in this category of disorders specific undegraded materials are stored in the lysosomes.
David A. Wenger, Paola Luzi, Mohammad A. Rafi   +2 more
doaj   +1 more source

A universal GlycoDesign for lysosomal replacement enzymes to improve circulation time and biodistribution

Frontiers in Bioengineering and Biotechnology, 2023
Currently available enzyme replacement therapies for lysosomal storage diseases are limited in their effectiveness due in part to short circulation times and suboptimal biodistribution of the therapeutic enzymes.
Yen-Hsi Chen, Yen-Hsi Chen, Weihua Tian, Weihua Tian, Makiko Yasuda, Zilu Ye, Zilu Ye, Ming Song, Ulla Mandel, Claus Kristensen, Lorenzo Povolo, André R. A. Marques, Tomislav Čaval, Tomislav Čaval, Albert J. R. Heck, Julio Lopes Sampaio, Ludger Johannes, Takahiro Tsukimura, Takahiro Tsukimura, Robert Desnick, Sergey Y. Vakhrushev, Zhang Yang, Zhang Yang, Henrik Clausen   +23 more
doaj   +1 more source

Molecular Mechanisms in Lysosomal Storage Diseases: From Pathogenesis to Therapeutic Strategies

Biomedicines, 2022
Lysosomal storage diseases (LSDs) are a group of metabolic diseases caused by inborn mutations of lysosomal enzymes, which lead to lysosome substrate accumulation in various cell types [...]
Valeria De Pasquale, Melania Scarcella, Luigi Michele Pavone   +2 more
doaj   +1 more source

Multiplex tandem mass spectrometry enzymatic activity assay for the screening and diagnosis of Mucolipidosis type II and III

Molecular Genetics and Metabolism Reports, 2023
Mucolipidosis type II and III (MLII/III) is caused by defects in the mannose-6-phosphate system, which is essential to target most of the lysosomal hydrolases to the lysosome.
Xinying Hong, Laura Pollard, Miao He, Michael H. Gelb, Timothy C. Wood   +4 more
doaj   +1 more source

Identification of active natural products that induce lysosomal biogenesis by lysosome-based screening and biological evaluation

Heliyon, 2022
Lysosomal biogenesis is an essential adaptive process by which lysosomes exert their function in maintaining cellular homeostasis. Defects in lysosomal enzymes and functions lead to lysosome-related diseases, including lysosomal storage diseases and ...
Xiao Ding, Xu Yang, Yueqin Zhao, Yinyuan Wang, Jimin Fei, Zhenpeng Niu, Xianxiang Dong, Xuenan Wang, Biao Liu, Hongmei Li, Xiaojiang Hao, Yuhan Zhao   +11 more
doaj   +1 more source

LYSOSOMAL ENZYMES AND THEIR RECEPTORS

Annual Review of Biochemistry, 1986
PERSPECTIVES AND SUMMARY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167 BIOSYNTHESIS OF LYSOSOMAL ENZyMES. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 Synthesis and Modifications in Endoplasmic Reticulum. . .. . . . . . .
Figura, Kurt von, Hasilik, Andrej
openaire   +3 more sources

Redox State and Lysosomal Activity in Women with Ovarian Cancer with Tumor Recurrence and Multiorgan Metastasis

Molecules, 2021
The aim of the study is to evaluate oxidant–antioxidant balance as well as lysosomal and anti-protease activities in ovarian cancer since it has been emphasized that the crucial inducing factor of carcinogenesis may be reactive oxygen/nitrogen species or,
Paweł Sutkowy, Jolanta Czuczejko, Bogdan Małkowski, Karolina Szewczyk-Golec, Rita Łopatto, Marta Maruszak, Alina Woźniak   +6 more
doaj   +1 more source

Role of lysosomal enzymes released by alveolar macrophages in the pathogenesis of the acute phase of hypersensitivity pneumonitis

Mediators of Inflammation, 1995
Hydrolytic enzymes are the major constituents of alveolar macrophages (AM) and have been shown to be involved in many aspects of the inflammatory pulmonary response.
J. L. Pérez-Arellano, M. N. Barrios, T. Martín, M. L. Sánchez, J. M. González Buitrago, A. Jiménez   +5 more
doaj   +1 more source

Inflammation-Involved Proteins in Blood Serum of Cataract Patients—A Preliminary Study

Biomedicines, 2023
Approximately 50% of all global blindness is caused by cataract in adults aged ≥50 years. The mechanisms of the disease are most arguably related to a redox imbalance and inflammation; therefore, the aim of the study was to evaluate the processes ...
Paweł Sutkowy, Hanna Lesiewska, Alina Woźniak, Grażyna Malukiewicz   +3 more
doaj   +1 more source

Reference values for lysosomal enzymes activities using dried blood spots samples - a Brazilian experience

Diagnostic Pathology, 2010
Background Lysosomal storage diseases (LSD) are inherited disorders caused by deficiency of lysosomal enzymes in which early diagnosis is essential to provide timely treatment. This study reports interval values for the activity of lysosomal enzymes that
Martins Ana M, Pereira Vanessa G, Rodrigues Mayra DB, Müller Karen B, D'Almeida Vânia   +4 more
doaj   +1 more source