Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease (2009)
Kielar, Catherine, Wishart, Thomas M., Palmer, Alice, Dihanich, Sybille, Wong, Andrew M., Macauley, Shannon L., ...
Neuronal ceroid lipofuscinoses (NCLs; Batten disease) are collectively the most frequent autosomal-recessive neurodegenerative disease of childhood, but the underlying cellular and molecular...
Von Schantz, Carina, Saharinen, Juha, Kopra, Outi, Cooper, Jonathan D, Gentile, Massimiliano, Hovatta, Iiris, ...
Abstract Background The neuronal ceroid lipofuscinoses (NCL) are a group of children's inherited neurodegenerative disorders, characterized by blindness, early dementia and pronounced cortical...
Kay, Gaham W., Palmer, David N., Rezaie, Payam, Cooper, Jonathan D.
The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited lysosomal storage diseases of children characterized by increasing blindness, seizures and profound neurodegeneration but...
Oswald, Manfred J., Palmer, David N., Kay, Graham W., Shemilt, Stephen J.A., Rezaie, Payam, Cooper, Jonathan D.
The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited neurodegenerative diseases characterized by gross brain atrophy, blindness, and intracellular accumulation of...
Pontikis, Charlie C., Cella, Claire V., Parihar, Nisha, Lim, Ming J., Chakrabarti, Shubhodeep, Mitchison, Hannah M., ...
Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in the hippocampus...
Chattopadhyay, Subrata, Ito, Masumi, Cooper, Jonathan D., Brooks, Andrew I., Curran, Timothy M., Powers, James M., ...
Mutations in the CLN3 gene are responsible for the neurodegenerative disorder Batten disease; however, the molecular basis of this disease remains unknown. In studying a mouse model for Batten...
Cooper, Jonathan D., Salehi, Ahmad, Delcroix, Jean-Dominique, Howe, Charles L., Belichenko, Pavel V., Chua-Couzens, Jane, ...
Age-related degeneration of basal forebrain cholinergic neurons (BFCNs) contributes to cognitive decline in Alzheimer's disease and Down's syndrome. With aging, the partial trisomy 16 (Ts65Dn) mouse...
Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues.
Heine, Claudia, Tyynelä, Jaana, Cooper, Jonathan D, Palmer, David N, Elleder, Milan, Kohlschütter, Alfried, ...
Neuronal ceroid lipofuscinosis type 6 and its sheep model (OCL6) are lysosomal storage disorders caused by mutations in the CLN6 gene product of unknown function. It has been proposed that...
Cooper, Jonathan D., Salehi, Ahmad, Delcroix, Jean-Dominique, Howe, Charles L., Belichenko, Pavel V., Chua-Couzens, Jane, ...
Age-related degeneration of basal forebrain cholinergic neurons (BFCNs) contributes to cognitive decline in Alzheimer's disease and Down's syndrome. With aging, the partial trisomy 16 (Ts65Dn) mouse...
Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues.
Heine, Claudia, Tyynelä, Jaana, Cooper, Jonathan D, Palmer, David N, Elleder, Milan, Kohlschütter, Alfried, ...
Neuronal ceroid lipofuscinosis type 6 and its sheep model (OCL6) are lysosomal storage disorders caused by mutations in the CLN6 gene product of unknown function. It has been proposed that...
Von Schantz, Carina, Saharinen, Juha, Kopra, Outi, Cooper, Jonathan D, Gentile, Massimiliano, Hovatta, Iiris, ...
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease
Kielar, Catherine, Wishart, Thomas M., Palmer, Alice, Dihanich, Sybille, Wong, Andrew M., Macauley, Shannon L., ...
Neuronal ceroid lipofuscinoses (NCLs; Batten disease) are collectively the most frequent autosomal-recessive neurodegenerative disease of childhood, but the underlying cellular and molecular...