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Journal of Neuroscience Research, ISSN 0360-4012, 11/2014, Volume 92, Issue 11, pp. 1591 - 1598
Journal Article
Pediatric Neurology, ISSN 0887-8994, 2017, Volume 69, pp. 102 - 112
Journal Article
Journal Article
Nature Reviews Neurology, ISSN 1759-4758, 03/2019, Volume 15, Issue 3, pp. 161 - 178
Batten disease (also known as neuronal ceroid lipofuscinoses) constitutes a family of devastating lysosomal storage disorders that collectively represent the... 
ENZYME-REPLACEMENT THERAPY | PRECLINICAL MOUSE MODEL | LATE-INFANTILE | TYPE-2 CLN2 DISEASE | LYSOSOMAL-STORAGE | NEURONAL-CEROID-LIPOFUSCINOSIS | LARGE ANIMAL-MODELS | BLOOD-BRAIN-BARRIER | DIRECTED GENE-THERAPY | OPTIC-NERVE DEGENERATION | CLINICAL NEUROLOGY
Journal Article
Neurobiology of Disease, ISSN 0969-9961, 07/2019, Volume 127, pp. 362 - 373
The late-infantile Batten disease or late-infantile neuronal ceroid lipofuscinosis (LINCL) is an autosomal recessive lysosomal storage disorder caused by... 
TPP1 | LINCL | PPARα | 3-Hydroxy-(2,2)-dimethyl butyrate | SURVIVAL | GEMFIBROZIL | PPAR alpha | NEUROSCIENCES | LIPID-LOWERING DRUG | CLN2 | ADOPTIVE TRANSFER | MOUSE MODEL | LYSOSOMAL-STORAGE | I ACTIVITY | GENE-TRANSFER | NEURONAL CEROID-LIPOFUSCINOSIS
Journal Article
PLoS ONE, ISSN 1932-6203, 08/2013, Volume 8, Issue 8, p. e73128
Journal Article
PLOS ONE, ISSN 1932-6203, 05/2017, Volume 12, Issue 5, p. e0176526
The Neuronal Ceroid Lipofuscinoses (NCLs), also known as Batten disease, result from mutations in over a dozen genes. Although, adults are susceptible, the... 
STORAGE | NEURONAL CEROID-LIPOFUSCINOSES | ATP SYNTHASE | GLIAL ACTIVATION | CODON READTHROUGH | MULTIDISCIPLINARY SCIENCES | BATTEN-DISEASE | READ-THROUGH | TRIPEPTIDYL-PEPTIDASE-I | LOW-LEVEL | SUBUNIT C | Serine Proteases - genetics | Neuronal Ceroid-Lipofuscinoses - therapy | Aminopeptidases - genetics | Male | Codon, Nonsense - physiology | Neuronal Ceroid-Lipofuscinoses - pathology | Mice, Knockout | Behavior, Animal | Gliosis - pathology | Animals | Neuronal Ceroid-Lipofuscinoses - genetics | Brain - pathology | Mice | Codon, Nonsense - genetics | Dipeptidyl-Peptidases and Tripeptidyl-Peptidases - genetics | Disease Models, Animal | Neuronal ceroid-lipofuscinosis | Care and treatment | Genetic aspects | Research | Gene mutations | Cerebellum | Brain | Disease | Genomics | Central nervous system | Cognitive ability | Nervous system | Chains (polymeric) | Assaying | Neuronal-glial interactions | Proteins | Enzymatic activity | Neurodegeneration | Diagnosis | Medical research | Enzymes | Neurodegenerative diseases | Lysergic acid diethylamide--LSD | Mortality | Amino acid sequence | Stop codon | Antisense oligonucleotides | Nucleic acids | Substrates | Membrane proteins | Polymerase chain reaction | Falls | Pathology | Storage | Stem cells | Death | Mutation | Protocol (computers) | Hippocampus | Drugs | Animal models | Physiological effects | Enzyme activity | Transcription | Epilepsy | Genes | Clinical trials | Aminoglycosides | Cortex (motor) | Reduction | Mucopolysaccharides | Attenuation | Aminoglycoside antibiotics | Children | Neuronal ceroid lipofuscinosis | Genotypes | Deposition | Age | Immune system | Colonies | CLN2 protein | Medicine | Brain research | Decay | In vivo methods and tests | LSD | Lysergic acid diethylamide
Journal Article
Brain, ISSN 0006-8950, 2013, Volume 136, Issue 5, pp. 1488 - 1507
Journal Article