Results 11 to 20 of about 929 (218)

Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy

Molecular Therapy: Methods & Clinical Development, 2017
Several lysosomal enzymes currently used for enzyme replacement therapy in patients with lysosomal storage diseases contain very low levels of mannose 6-phosphate, limiting their uptake via mannose 6-phosphate receptors on the surface of the deficient ...
Lin Liu, Wang-Sik Lee, Balraj Doray, Stuart Kornfeld   +3 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

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

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

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

Lysosomal enzymes and mannose 6-phosphate receptors in the lacrimal drainage system: Evidence and its potential implications

Indian Journal of Ophthalmology, 2018
Purpose: To investigate the presence and patterns of lysosomal enzymes and mannose 6-phosophate receptor (MPRs) in human lacrimal drainage system. Methods: The study was performed on healthy lacrimal sacs and nasolacrimal ducts obtained from exenteration
Mohammad Javed Ali, Ashapogu Venugopal, Kavyashree Sakharayapatna Ranganath, Nadimpalli Siva Kumar   +3 more
doaj   +1 more source

Newborn Screening for Krabbe Disease—Illinois Experience: Role of Psychosine in Diagnosis of the Disease

International Journal of Neonatal Screening, 2021
Population-based newborn screening for Krabbe disease was initiated by measurement of galactocerebrosidase (GALC) activity in the state of Illinois in December 2017.
Khaja Basheeruddin, Rong Shao, Fran Balster, Pearlie Gardley, Laura Ashbaugh   +4 more
doaj   +1 more source

Development of Organelle Replacement Therapy Using a Stearyl-Polyhistidine Peptide against Lysosomal Storage Disease Cells

Molecules, 2019
We previously reported on a polyhistidine peptide, His16 peptide, as a new cell-penetrating peptide. This peptide is anticipated to be a new carrier for drug delivery systems (DDSs) for targeting intracellular lysosomes because it can transport ...
Taiki Hayashi, Riku Okamoto, Tsuyoshi Kawano, Takashi Iwasaki   +3 more
doaj   +1 more source

The activity of lysosomal hydrolases (proteases and nucleases) in salmon (Salmo salar L.) juveniles of different age categories from the Indera River

Transactions of the Karelian Research Centre of the Russian Academy of Sciences, 2016
The results on the activity of lysosomal hydrolases (proteases (cathepsins) and nucleases) in salmon (Salmo salar L.) underyearlings (0+), 1+ and 2+ parr, and smolts (2+, 3+)from the Indera River (White Sea drainage basin) are reported, testifying their ...
Marina Krupnova, Elizaveta Vdovichenko, Rimma Vysotskaya, Denis Efremov, Aleksey Veselov, Nina Nemova   +5 more
doaj   +1 more source

Conformational dynamics of free and membrane-bound human Hsp70 in model cytosolic and endo-lysosomal environments

Communications Biology, 2021
Calvaresi et al. use hydrogen-deuterium exchange coupled to mass spectrometry to compare the conformational dynamics of the human heat shock protein Hsp70 when free and when associated with liposomes composed of common lysosomal lipids.
Valeria Calvaresi, Line T. Truelsen, Sidsel B. Larsen, Nikolaj H. T. Petersen, Thomas Kirkegaard, Kasper D. Rand   +5 more
doaj   +1 more source