PMID:12020865

Citation	Bolivar, VJ, Scott Ganus, J and Messer, A (2002) The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse. Brain Res. 937:74-82
Abstract	The motor neuron degeneration (mnd) mouse, which has widespread abnormal accumulating lipoprotein and neuronal degeneration, has a mutation in CLN8, the gene for human progressive epilepsy with mental retardation (EPMR). EPMR is one of the neuronal ceroid lipofuscinoses (NCLs), a group of neurological disorders characterized by autofluorescent lipopigment accumulation, blindness, seizures, motor deterioration, and dementia. The human phenotype of EPMR suggests that, in addition to the motor symptoms previously categorized, various types of progressive behavioral abnormalities would be expected in mnd mice. We have therefore examined exploratory behavior, fear conditioning, and aggression in 2-3 month and 4-5 month old male mnd mice and age-matched C57BL/6 (B6) controls. The mnd mice displayed increased activity with decreased habituation in the activity monitor, poor contextual and cued memory, and heightened aggression relative to B6 controls. These behavioral deficits were most prominent at 4-5 months of age, which is prior to the onset of gross motor symptoms at 6 months. Our results provide a link from the mutation via pathology to a quantifiable multidimensional behavioral phenotype of this naturally occurring mouse model of NCL.
Links	PubMed
Keywords	Aggression/physiology; Animals; Behavior, Animal/physiology; Brain/pathology; Conditioning, Classical/physiology; Disease Models, Animal; Disease Progression; Exploratory Behavior/physiology; Fear/physiology; Habituation, Psychophysiologic/genetics; Habituation, Psychophysiologic/physiology; Male; Membrane Proteins/deficiency; Membrane Proteins/genetics; Membrane Proteins/physiology; Memory Disorders/genetics; Memory Disorders/physiopathology; Mice; Mice, Inbred C57BL; Mice, Neurologic Mutants/genetics; Mice, Neurologic Mutants/psychology; Motor Neuron Disease/genetics; Motor Neuron Disease/pathology; Motor Neuron Disease/psychology; Myoclonic Epilepsies, Progressive/genetics; Myoclonic Epilepsies, Progressive/pathology; Myoclonic Epilepsies, Progressive/psychology; Nerve Tissue Proteins/deficiency; Nerve Tissue Proteins/genetics; Nerve Tissue Proteins/physiology; Neuronal Ceroid-Lipofuscinoses/genetics; Neuronal Ceroid-Lipofuscinoses/pathology; Neuronal Ceroid-Lipofuscinoses/psychology; Phenotype
edit table

Annotations

Gene product	Qualifier	GO ID	GO term name	Evidence Code	with/from	Aspect	Notes	Status
edit table

References

See Help:References for how to manage references in GONUTS.