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Juvenile Neuronal Ceroid Lipofuscinosis

Known as: Juvenile Cerebroretinal Degeneration, Batten-Mayou Disease, Cerebroretinal Degenerations, Juvenile 
A condition associated with mutation(s) in the CLN3 gene, encoding battenin. The condition is one of a group of genetically heterogeneous… 
National Institutes of Health

Related topics

Related topics

20 relations
Abnormality of extrapyramidal motor functionAdult Neuronal Ceroid LipofuscinosisAutosomal recessive inheritanceBehavioral change

Broader (2)

Cerebral lipidosesNeuronal Ceroid-Lipofuscinoses

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2004
Highly Cited
2004
Mechanism of Activity-Dependent Downregulation of the Neuron-Specific K-Cl Cotransporter KCC2
GABA-mediated fast-hyperpolarizing inhibition depends on extrusion of chloride by the neuron-specific K-Cl cotransporter, KCC2… 
Highly Cited
1999
Highly Cited
1999
Reeler/Disabled-like Disruption of Neuronal Migration in Knockout Mice Lacking the VLDL Receptor and ApoE Receptor 2
Highly Cited
1995
Highly Cited
1995
Isolation of a novel gene underlying batten disease, CLN3
Highly Cited
1995
Highly Cited
1995
Neuronal pathfinding is abnormal in mice lacking the neuronal growth cone protein GAP-43
Highly Cited
1993
Highly Cited
1993
Comparison of the Saccharomyces cerevisiae G1 cyclins: Cln3 may be an upstream activator of Cln1, Cln2 and other cyclins.
In the budding yeast Saccharomyces cerevisiae, the G1 cyclins Cln1, Cln2 and Cln3 regulate entry into the cell cycle (Start) by… 
Review
1992
Review
1992
Neuronal Ca2+/calmodulin-dependent protein kinases.
Widespread localization, responsiveness to numerous signal transduction systems, and broad substrate specificity enable the… 
Review
1989
Review
1989
Neurotransmitter regulation of neuronal outgrowth, plasticity and survival
Highly Cited
1985
Highly Cited
1985
Plasticity of hippocampal circuitry in Alzheimer's disease.
Two markers of neuronal plasticity were used to compare the response of the human central nervous system to neuronal loss… 
Highly Cited
1978
Highly Cited
1978
Neuronal plasticity in the limbic system during classical conditioning of the rabbit nictitating membrane response. I. The hippocampus
Highly Cited
1978
Highly Cited
1978
ABNORMAL NEURONAL MIGRATION, DERANGED CEREBRAL CORTICAL ORGANIZATION, AND DIFFUSE WHITE MATTER ASTROCYTOSIS OF HUMAN FETAL BRAIN: A MAJOR EFFECT OF METHYLMERCURY POISONING IN UTERO
Detailed clinical and neuropathological studies have been made in two fullterm newborn human infants who were exposed to… 
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