Results 41 to 50 of about 6,754 (244)
Advanced Science, EarlyView.Nanomaterials offer dual applications in allergy management. For diagnosis, nanomaterials enhance analytical sensitivity and improve detection in specific IgE and functional assays such as the basophil activation test (BAT). For allergen‐specific immunotherapy, nanomaterials enable allergen masking and controlled release, and effectively modulate the ...
Madiha Habib +8 more
wiley +1 more source
Neurobiology of Disease, 2017 Batten disease (neuronal ceroid lipofuscinosis) refers to a group of neurodegenerative lysosomal storage diseases predominantly affecting children. There are currently no effective treatments, and the functions of many of the associated gene products are
Hannah L Best +5 more
doaj +1 more source
Frontiers in Genetics, 2021 The neuronal ceroid lipofuscinoses (NCLs), also known as Batten disease, are a group of autosomal recessive lysosomal storage disorders that are characterized by neurodegeneration, progressive cognitive decline, motor impairment, ataxia, loss of vision ...
Paschalis Nicolaou +13 more
doaj +1 more source
A Knock-In Reporter Model of Batten Disease [PDF]
The Journal of Neuroscience, 2007 Juvenile neuronal ceroid lipofuscinosis is a severe inherited neurodegenerative disease resulting from mutations inCLN3(ceroid-lipofuscinosis, neuronal 3, juvenile). CLN3 function, and where and when it is expressed during development, is not known. In this study, we generated a knock-in reporter mouse to elucidate CLN3 expression during embryogenesis ...
Steven L, Eliason +6 more
openaire +2 more sources
Adipocyte Myoglobin Is a Determinant of Energy Expenditure and a Potential Target to Limit Obesity
Advanced Science, EarlyView.Myoglobin, known as a muscle oxygen‐carrying protein, is shown to play a key role in fat cells that burn energy. Loss of myoglobin reduces the body's ability to generate heat and increases obesity risk, while restoring it improves metabolism. The study identifies myoglobin as a regulator of fat burning and a potential target to enhance energy ...
Christian Strehlau +22 more
wiley +1 more source
Cardiac magnetic resonance findings in neuronal ceroid lipofuscinosis: A case report
Frontiers in Neurology, 2022 Cardiac magnetic resonance imaging (MRI) is an essential tool for the study of hypertrophic cardiomyopathies (HCM) and for differentiating HCM from conditions with increased ventricular wall thickness, such as cardiac storage diseases.
Giancarlo Todiere +7 more
doaj +1 more source
Slc44a2 Deficiency Unveils an IFN‐I–Dependent Feedback Control of pDC Egress
Advanced Science, EarlyView.Working model of SLC44A2‐mediated maintenance of pDC homeostasis. This model illustrates two central mechanisms by which SLC44A2 regulates pDC homeostasis: (1) SLC44A2 limits IFN‐I production by exporting amino acids (T, N, Q), thereby preventing spontaneous pDC activation.
Ruiqun Chen +11 more
wiley +1 more source
Advanced Science, EarlyView.Chronic microinflammation in high myopia suppresses MANF in lens epithelial cells. MANF normally resides within MAMs and promotes ubiquitin‐mediated degradation of the ER Ca2+ pump SERCA2. MANF loss causes pathological SERCA2 accumulation, MAM hyperassembly, and disrupted ER‐to‐mitochondria Ca2+ transfer, leading to mitochondrial failure, oxidative ...
Xin Liu +8 more
wiley +1 more source
Discovery of a CLN7 model of Batten disease in non-human primates
Neurobiology of Disease, 2018 We have identified a natural Japanese macaque model of the childhood neurodegenerative disorder neuronal ceroid lipofuscinosis, commonly known as Batten Disease, caused by a homozygous frameshift mutation in the CLN7 gene (CLN7−/−).
Jodi L. McBride +15 more
doaj +1 more source
American Journal of Botany, EarlyView.Abstract Premise Climate change is altering the timing of many plant phenological events, yet most studies focus on flowering, with less attention given to later reproductive stages such as fruit development and ripening. Vaccinium angustifolium (lowbush blueberry) is an important perennial berry species that may be sensitive to these changes.
Jenna Floyd +5 more
wiley +1 more source