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of 4
pro vyhledávání: '"Lucy A. Barry"'
Autor:
Lucy Anne Barry, Graham William Kay, Nadia Lesley Mitchell, Samantha Jane Murray, Nigel P Jay, David Norris Palmer
Publikováno v:
PLoS ONE, Vol 17, Iss 4, p e0261544 (2022)
The neuronal ceroid lipofuscinoses (NCLs; Batten disease) are fatal, mainly childhood, inherited neurodegenerative lysosomal storage diseases. Sheep affected with a CLN6 form display progressive regionally defined glial activation and subsequent neur
Externí odkaz:
https://doaj.org/article/fda9be08b97543b6bbeacd092c48cbf7
Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network
Autor:
Nadia L. Mitchell, Jarol Z. Chen, Peter J. Houweling, David Palmer, Chia-Tien Chang, Lucy A. Barry, Imke Tammen, Nicole J. Neverman, Stephanie M. Hughes
Publikováno v:
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1852:2279-2286
Studies on naturally occurring New Zealand and Australian ovine models of the neuronal ceroid-lipofuscinoses (Batten disease, NCLs) have greatly aided our understanding of these diseases. Close collaborations between the New Zealand groups at Lincoln
Publikováno v:
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease; Vol 1832
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease
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 accum
Autor:
Stephanie M. Hughes, Kathryn S. Linterman, Michael A. Black, Nadia L. Mitchell, Graham W. Kay, Mark S. Sands, David Palmer, Robin G. McFarlane, Lucy A. Barry
Publikováno v:
Human Gene Therapy. 22:1011-1020
The neuronal ceroid lipofuscinoses (NCLs; Batten disease) are inherited neurodegenerative lysosomal storage diseases with common clinical features of blindness and seizures culminating in premature death. Gene-therapy strategies for these diseases de