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Biochimica Et Biophysica Acta - Molecular Basis of Disease, 2013 In childhood the neuronal ceroid lipofuscinoses (NCL) are the most frequent lysosomal diseases and the most frequent neurodegenerative diseases but, in adulthood, they represent a small fraction among the neurodegenerative diseases. Their morphology is marked by: (i) loss of neurons, foremost in the cerebral and cerebellar cortices resulting in ...
Alessandro Simonati
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NCL diseases — clinical perspectives [PDF]
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2013 The neuronal ceroid lipofuscinoses (NCLs) are lysosomal storage disorders and together are the most common degenerative brain diseases in childhood. They are a group of disorders linked by the characteristic accumulation of abnormal storage material in neurons and other cell types, and a degenerative disease course.
Schulz A. +4 more
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Human NCL Neuropathology [PDF]
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2015 The neuronal ceroid lipofuscinoses (NCL) currently encompass fourteen genetically different forms, CLN1 to CLN14, but are all morphologically marked by loss of nerve cells, particularly in the cerebral and cerebellar cortices, and the cerebral and extracerebral formation of lipopigments.
Radke, Josefine +2 more
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A rewriting logic semantics for NCL
Science of Computer Programming, 2015 The Nested Context Language (NCL) is a multimedia authoring language that is part of the standard for digital television in Brazil and in Latin America and the ITU standard for IPTV services. To properly support authors in the development of NCL applications, it is important to provide tools with validation capabilities.
Joel dos Santos +2 more
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A Non‐G‐Quadruplex DNA Aptamer Targeting NCL for Diagnosis and Therapy in Bladder Cancer
Advanced Healthcare Materials, 2023 Bladder cancer (BC) is a highly aggressive malignant tumor affecting the urinary system, characterized by metastasis and a poor prognosis that often leads to limited therapeutic success.
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Niclosamide-loaded nanoparticles (Ncl-NPs) reverse pulmonary fibrosis in vivo and in vitro
Journal of Advanced Research, 2023 Introduction: Idiopathic pulmonary fibrosis (IPF), a life-threatening interstitial lung disease, is characterized by excessive activation and proliferation of fibroblasts and epithelial-mesenchymal transition (EMT) of alveolar epithelial cells (AEC ...
Liang Ouyang +2 more
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Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2013 Despite the identification of a large number of disease-causing genes in recent years, it is still unclear what disease mechanisms operate in the neuronal ceroid lipofuscinoses (NCLs, Batten disease). As a group they are defined by the specific accumulation of protein, either subunit c of mitochondrial ATP synthase or SAPs A and D in lysosome-derived ...
Palmer, DN +3 more
openaire +4 more sources
NCL++: Nested Collaborative Learning for Long-Tailed Visual Recognition [PDF]
Pattern Recognition, 2023 Long-tailed visual recognition has received increasing attention in recent years. Due to the extremely imbalanced data distribution in long-tailed learning, the learning process shows great uncertainties. For example, the predictions of different experts
Zichang Tan +5 more
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Cell biology of the NCL proteins: What they do and don't do
Biochimica Et Biophysica Acta - Molecular Basis of Disease, 2015 David Pearce, Attila Kovacs
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Frontiers in Immunology, 2023 Objectives The tumor microenvironment (TME) play important roles in progression of endometrial carcinoma (EC). We aimed to assess the cell populations in TME of EC.
Xinnian Yu +5 more
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