Cln^Δex7/8 knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth

Keyphrases

• Knock-in Mouse Model 100%
• Lipofuscinosis 100%
• Progressive Neurological Disease 100%
• Juvenile-onset 100%
• Central Nervous System 80%
• CLN3 60%
• Premature Death 20%
• Cerebellum 20%
• Liver 20%
• Retina 20%
• Cerebral Cortex 20%
• Alternative Splicing 20%
• Neurological Deficit 20%
• Per Se 20%
• Accruals 20%
• Recessive Inheritance 20%
• Homozygote 20%
• Liver Enzymes 20%
• Neuronal Population 20%
• Genomic DNA (gDNA) 20%
• Harmful Impact 20%
• Degenerative Changes 20%
• Truncated Protein 20%
• Batten Disease 20%
• Truncated mutant 20%
• DNA Deletion 20%
• Central Nervous System Development 20%
• Gene Encoding 20%
• Disease Features 20%
• An-1 20%
• Membrane Deposition 20%

Biochemistry, Genetics and Molecular Biology

• CLN3 100%
• Central Nervous System Neurons 100%
• Enzyme 50%
• Allele 50%
• Nervous System Development 50%
• Recessive Inheritance 50%
• Homozygote 50%
• Batten Disease 50%
• Neuronal Ceroid Lipofuscinosis 50%
• Genomics 50%

Neuroscience

• Central Nervous System 100%
• Nervous System Disorder 100%
• Central Nervous System Neurons 66%
• Cerebellum 33%
• Cerebral Cortex 33%
• Neurogenesis 33%
• Genomic DNA 33%
• Neuronal Ceroid Lipofuscinosis 33%
• Liver Enzyme 33%