Results 121 to 130 of about 677,727 (291)

Effectiveness and tolerability of migalastat in adult Fabry disease: A single regional centre experience

Molecular Genetics and Metabolism Reports
Background: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by deficient α-galactosidase A (α-Gal) activity, leading to progressive renal, cardiac, and cerebrovascular involvement.
Eamon P. McCarron, Rajkumar Chinnadurai, Karolina M. Stepien, John Bassett, Jonathan Meyer, Reena Sharma, Peter Woolfson, Ana Jovanovic   +7 more
doaj   +1 more source

Preharvest supplemental lighting enhances productivity and modulates postharvest quality in hydroponically grown Apulian unripe melon ‘Scopatizzo’ (Cucumis melo L.)

Journal of the Science of Food and Agriculture, EarlyView.
Abstract BACKGROUND Preharvest light management is increasingly used to enhance yield and fruit quality in soilless systems, yet its effects on unripe melon fruits remain poorly understood. This study evaluated the impact of supplemental red–blue light‐emitting diode (LED) lighting applied during cultivation in a closed‐cycle hydroponic system on ...
Onofrio Davide Palmitessa, Marica Troilo, Carlo Porfido, Roberto Terzano, Davide De Angelis, Massimiliano Renna, Francesco Caponio, Pietro Santamaria   +7 more
wiley   +1 more source

Catalytic Mechanism of Human alpha-Galactosidase

, 2010
The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL A; E.C. 3.2.1.22) is responsible for the breakdown of alpha-galactosides in the lysosome. Defects in human alpha-GAL lead to the development of Fabry disease, a lysosomal storage disorder
Clark, Nathaniel E., Garman, Scott C., Guce, Abigail I., Kulminskaya, Anna A., Salgado, Eric N., Ivanen, Dina R., Brumer, Harry,   +6 more
core   +1 more source

Rare‐Variant Burden across Lysosomal Genes Implicates Sialylation and Ganglioside Metabolism in Parkinson's Disease

Movement Disorders, EarlyView.
Abstract Background Lysosomal dysfunction is central to Parkinson's disease (PD) pathogenesis, with GBA1 representing the strongest established genetic risk factor. Numerous other genes involved in lysosomal sphingolipid, glycosphingolipid, and ceramide metabolism have been proposed as contributors to PD, highlighting the need for genetic analyses ...
Konstantin Senkevich, Sitki Cem Parlar, Cloe Chantereault, Lang Liu, Eric Yu, Uladzislau Rudakou, Jamil Ahmad, Jennifer A. Ruskey, Farnaz Asayesh, Dan Spiegelman, Cheryl Waters, Oury Monchi, Yves Dauvilliers, Nicolas Dupré, Lior Greenbaum, Sharon Hassin‐Baer, Irina Miliukhina, Alla Timofeeva, Anton Emelyanov, Sofya Pchelina, Roy N. Alcalay, Ziv Gan‐Or   +21 more
wiley   +1 more source

Purification of alpha-galactosidase from pepino (Solanum muricatum) by three-phase partitioning

, 2011
Three-phase partitioning (TPP) is a bioseparation technique used for the extraction, concentration and purification of biomolecules. The technique consists of simultaneous addition of a salt (generally ammonium sulfate) to the crude extract followed by ...
Sen, Aysu, Aysu Şen, Hasan Bayraktar, Seçil Önal, Eryilmaz, Melike, Onal, Secil, Bayraktar, Hasan, Melike Eryılmaz   +7 more
core   +1 more source

The N‐Glycome to Differentiate Mesenchymal Stem Cells Upon Chondrogenic Differentiation, Dedifferentiation, and Senescence

PROTEOMICS, EarlyView.
ABSTRACT Mesenchymal stem cells (MSCs) are adult stem cells able to self‐renewal or differentiation into different cell types, including chondrocytes. N‐Glycans are post‐translational modifications of glycoproteins that contribute to vital cell functions.
Houda Montacir, Karina Biskup, Michael Sittinger, Rudolf Tauber, Véronique Blanchard   +4 more
wiley   +1 more source

HvarAKR1B1‐mediated tolerance in Hippodamia variegata: Deciphering the metabolic adaptation and fitness costs under prolonged starvation

Pest Management Science, EarlyView.
Starvation stress significantly affected the growth and development of Hippodamia variegata. Combined transcriptome and metabolome analysis and RNA interference verification revealed that HvarAKR1B1 may be involved in the response of Hippodamia variegata to starvation stress.
Bing‐mei Song, De‐qin Hu, Yuan Li, Wang‐quan Jiao, Xiao‐li Ma, Hong‐sheng Pan   +5 more
wiley   +1 more source

Peptide‐based antibacterial nanoplatforms: Design principles, stimuli‐regulated behaviors, and applications

Responsive Materials, EarlyView.
Peptide‐based antibacterial nanoplatforms, encompassing self‐assembled peptides and peptide‐engineered inorganic, polymeric, and lipid nanocarriers, are systematically reviewed. The article highlights design principles and stimuli‐responsive regulation for improving peptide stability, delivery efficiency, and antibacterial performance.
Peng Tan, Chenlong Zhou, Shuaikang Yang, Zhenduo Chen, Guanghui Zhao, Qi Tang, Haoyu Yan, Feng Li, Xi Ma   +8 more
wiley   +1 more source

Dietary α-galactosidase and xylanase to improve the nutritional value of corn-soybean-rapeseed meal diets in broiler chickens

Journal of Applied Animal Research
Two experiments were performed to assess the effects of an enzyme preparation (containing α-galactosidase and xylanase) on chicken performance, carcass traits, footpad dermatitis score, and nutrient digestibility.
Alberto Viñado, Saksit Srinongkote, Josep Mascarell, Rene Morales, Javier Estévez, Sergi Carné   +5 more
doaj   +1 more source

Assessment of the feed additive consisting of alpha-galactosidase produced by Saccharomyces cerevisiaeCBS 615.94 and endo-1,4-beta-glucanase produced by Aspergillus nigerCBS 120604 (Agal-Pro BL/BL-L®) for use in chickens for fattening, minor poultry species for fattening and chickens reared for laying for the renewal of its authorisation (Kerry Ingredients & Flavours Ltd.). [PDF]

EFSA J, 2023
EFSA Panel on Additives and Products or Substances used in Animal Feed (FEEDAP), Bampidis V, Azimonti G, Bastos ML, Christensen H, Dusemund B, Durjava M, Kouba M, López-Alonso M, López Puente S, Marcon F, Mayo B, Pechová A, Petkova M, Ramos F, Ramos F, Sanz Y, Villa RE, Woutersen R, Anguita M, Brozzi R, Ortuño J, Pettenati E, Tarrés-Call J, García-Cazorla Y.   +24 more
europepmc   +1 more source