Associated Papers
CLN6 deficiency causes selective changes in the lysosomal protein composition.
Tuermer A et al.
Proteomics, 2021
Gene Therapy Corrects Brain and Behavioral Pathologies in CLN6-Batten Disease.
Cain JT et al.
Molecular Therapy : the Journal of the American Society of Gene Therapy, 2019
Apoptotic photoreceptor loss and altered expression of lysosomal proteins in the nclf mouse model of neuronal ceroid lipofuscinosis.
Bartsch U et al.
Investigative Ophthalmology & Visual Science, 2013
Progressive retinal degeneration and glial activation in the CLN6 (nclf) mouse model of neuronal ceroid lipofuscinosis: a beneficial effect of DHA and curcumin supplementation.
Mirza M et al.
Plos One, 2013
A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.
Morgan JP et al.
Plos One, 2013
Opening Pandora's jar: a primer on the putative roles of CRMP2 in a panoply of neurodegenerative, sensory and motor neuron, and central disorders.
Khanna R et al.
Future Neurology, 2012
High expression of disease-related Cln6 in the cerebral cortex, purkinje cells, dentate gyrus, and hippocampal ca1 neurons.
Thelen M et al.
Journal of Neuroscience Research, 2012
Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.
Thelen M et al.
Plos One, 2012
Pathogenic mutations cause rapid degradation of lysosomal storage disease-related membrane protein CLN6.
Kurze AK et al.
Human Mutation, 2010
Neuronal ceroid lipofuscinoses.
Jalanko A et al.
Biochimica Et Biophysica Acta, 2009
Molecular genetics of the NCLs -- status and perspectives.
Siintola E et al.
Biochimica Et Biophysica Acta, 2006
The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein.
Wheeler RB et al.
American Journal of Human Genetics, 2002
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