Fingolimod and Teriflunomide Attenuate Neurodegeneration in Mouse Models of Neuronal Ceroid Lipofuscinosis
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چکیده
منابع مشابه
With Neuronal Ceroid Lipofuscinosis
An 11-year-old girl with juvenile neuronal ceroid lipofuscinosis developed acute pancreatitis secondary to valproic acid treatment, which resolved after discontinuation of the medication and conservative treatment. To my knowledge, this is the first reported case of neuronal ceroid lipofuscinosis with valproate-associated pancreatitis. Children with neuronal ceroid lipofuscinosis may be suscept...
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Juvenile neuronal ceroid lipofuscinoses (JNCL), commonly known as Batten disease, is a progressive neurodegenerative disorder of childhood characterized by blindness, seizures, motor and cognitive decline, leading to death in early adulthood. Mutations within the CLN3 gene, which encodes a putative lysosomal protein of unknown function, are the underlying cause of JNCL. Over 85% of JNCL patient...
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A lysosomal storage disease with accumulation of periodic acid-Schiff- and Sudan black-positive autofluorescent granules in neurons occurred in one 5 1/2- and one 7-year-old dachshund. Ultrastructurally, the storage material consisted of membranous material arranged in stacks and fingerprint patterns. The disease was defined as ceroid-lipofuscinosis, and resembled a previously reported case in ...
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The neuronal ceroid-lipofuscinoses are a group of diseases characterized by progressive accumulation of autofluorescent lipopigments within neurons with associated neuronal damage." Variations of these diseases occur which include accumulations of lipopigments in viscera. The diseases long have been recognized in man where earlier studies of the pathogenesis suggested an increased rate of perox...
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The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative diseases characterised by the accumulation of autofluorescent lipopigment in a variety of tissues. At least six different forms of NCL occur in humans and the disease has been identified in various animal species, including sheep. Clinical features of NCL are dementia, loss of vision, motor disturbances, and pr...
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ژورنال
عنوان ژورنال: Molecular Therapy
سال: 2017
ISSN: 1525-0016
DOI: 10.1016/j.ymthe.2017.04.021