Results 61 to 70 of about 6,754 (244)

Neuronal Ceroid Lipofuscinosis in a Domestic Cat Associated with a DNA Sequence Variant That Creates a Premature Stop Codon in CLN6

G3: Genes, Genomes, Genetics, 2020
A neutered male domestic medium-haired cat presented at a veterinary neurology clinic at 20 months of age due to progressive neurological signs that included visual impairment, focal myoclonus, and frequent severe generalized seizures that were ...
Martin L. Katz, Reuben M. Buckley, Vanessa Biegen, Dennis P. O’Brien, Gayle C. Johnson, Wesley C. Warren, Leslie A. Lyons   +6 more
doaj   +1 more source

Pectinase‐Based Bioprocesses for Circular Bioeconomy and Sustainable Industrial Development

Biotechnology and Bioengineering, EarlyView.
This picture demonstrates a unified strategy for sustainable pectinase production, focusing on microbial bioproduction, innovative strategies in enzyme engineering (Protein engineering, rational enzyme design, directed evolution and heterologous expression) and various applications in industry, including food, textiles, pulp and paper, agriculture and ...
Nisha Sharma, Dimple Tanwar, Usha Grewal, Murugesan Mohana Keerthi, Yogesh K. Ahlawat, Nitin Pandey, Chirag G. Makvana   +6 more
wiley   +1 more source

The CLN3 gene and protein: What we know

Molecular Genetics & Genomic Medicine, 2019
Background One of the most important steps taken by Beyond Batten Disease Foundation in our quest to cure juvenile Batten (CLN3) disease is to understand the State of the Science. We believe that a strong understanding of where we are in our experimental
Myriam Mirza, Anna Vainshtein, Alberto DiRonza, Uma Chandrachud, Luke J. Haslett, Michela Palmieri, Stephan Storch, Janos Groh, Niv Dobzinski, Gennaro Napolitano, Carolin Schmidtke, Danielle M. Kerkovich   +11 more
doaj   +1 more source

Repurposing of tamoxifen ameliorates CLN3 and CLN7 disease phenotype

EMBO Molecular Medicine, 2021
Batten diseases (BDs) are a group of lysosomal storage disorders characterized by seizure, visual loss, and cognitive and motor deterioration. We discovered increased levels of globotriaosylceramide (Gb3) in cellular and murine models of CLN3 and CLN7 ...
Chiara Soldati, Irene Lopez‐Fabuel, Luca G Wanderlingh, Marina Garcia‐Macia, Jlenia Monfregola, Alessandra Esposito, Gennaro Napolitano, Marta Guevara‐Ferrer, Anna Scotto Rosato, Einar K Krogsaeter, Dominik Paquet, Christian M Grimm, Sandro Montefusco, Thomas Braulke, Stephan Storch, Sara E Mole, Maria A De Matteis, Andrea Ballabio, Julio L Sampaio, Tristan McKay, Ludger Johannes, Juan P Bolaños, Diego L Medina   +22 more
doaj   +1 more source

Characterization of Immune Responses to RBD‐SD1 Nanoparticle Vaccines Derived From ACE2‐Capable HKU5‐CoV‐2, NeoCoV, and HKU5 Merbecoviruses

Biotechnology and Bioengineering, EarlyView.
SpyCatcher‐mi3 nanoparticles presenting HKU5, NeoCoV, and HKU5‐CoV‐2 RBD‐SD1 induced strong, cross‐reactive IgG responses in immunized mice. The surrogate virus neutralization test demonstrated strong activity in sera from homologous vaccination. ABSTRACT Recent discoveries of merbecoviruses such as HKU5, NeoCoV, and HKU5‐CoV‐2 that use angiotensin ...
Peter J. Halfmann, Jeong Soo Lee, Augustine Duffy, Tong Wang, Bingcheng Huang, James Z. Kurdi, Ajay Inampudi, Natasha Chen, Elizabeth Frimpong, Carmen Rai, Yoshihiro Kawaoka, Ravi S. Kane   +11 more
wiley   +1 more source

How wildlife respond to tropical cyclones: short‐term tactics and long‐term impacts

Biological Reviews, EarlyView.
ABSTRACT From butterflies to lizards and from sharks to seabirds, wildlife exhibit tactics to survive the impacts of tropical cyclones, also known as hurricanes, cyclones, or typhoons depending on where they occur. Some species seek refuge during the storm by moving, some remain in place and ride it out, and others move longer distances, avoiding the ...
Erin L. Koen, Mohamed Khalil Meliane, Zachery B. Holmes, Karl E. Miller, William J. Barichivich, Emilie Dedeban, Alex Furst, Miranda Imeri, Peyton E. Niebanck, Samantha Nunn, Kailee Pearson, Nicole Rita, Brier Ryver, Dakotah Shaffer, Susan C. Walls, E. Hance Ellington   +15 more
wiley   +1 more source

Morphometric Brain Changes in a Merino Sheep (Ovis aries) CLN6 Neuronal Ceroid Lipofuscinosis Model

Biology
The neuronal ceroid lipofuscinoses are the most common group of human paediatric genetic neurodegenerative disorders and have also been reported in multiple animal species.
Amelia Nanni, Emma Elcombe, Maverick Ho Ming Cheung, Timothy Stait-Gardner, Marina Gimeno, Imke Tammen, Marianne D. Keller   +6 more
doaj   +1 more source

Flight of the dragons: a global review of migration in Odonata

Biological Reviews, EarlyView.
ABSTRACT Insects are the most abundant and ecologically important animal migrants. Yet, we know relatively little about the patterns and processes underlying insect migration. Dragonflies (Anisoptera) and damselflies (Zygoptera) comprise the ancient insect order Odonata, whose ancestors were the first organisms to fly on Earth.
Johanna S.U. Hedlund, Myles Menz, Jason W. Chapman, Alexander Hayward   +3 more
wiley   +1 more source

Modelling of Neuronal Ceroid Lipofuscinosis Type 2 in Dictyostelium discoideum Suggests That Cytopathological Outcomes Result from Altered TOR Signalling

Cells, 2019
The neuronal ceroid lipofuscinoses comprise a group of neurodegenerative disorders with similar clinical manifestations whose precise mechanisms of disease are presently unknown.
Paige K. Smith, Melodi G. Sen, Paul R. Fisher, Sarah J. Annesley   +3 more
doaj   +1 more source

Humans are not unique: difficult birth is common in placental mammals

Biological Reviews, EarlyView.
ABSTRACT Human childbirth is widely presumed to be uniquely difficult and dangerous compared to birth in other mammals. Tight fetopelvic proportions can result in obstructed labour and contribute to high rates of maternal and neonatal mortality. Ideas summarised under the ‘obstetrical dilemma’ have contributed to this assumption by explaining difficult
Nicole D. S. Grunstra
wiley   +1 more source