Primary neurologic screening and motor coordination of Dstdt-J mutant mice (dystonia musculorum) with spinocerebellar atrophy

The autosomal recessive dystonia musculorum (Dst(dt-J)) mutation causes degenerative lesions of peripheral and central sensory pathways. A test battery of motor, sensory, postural, and autonomic functions was used to compare young control and homozygous Dst(dt-J) mice. The Dst(dt-J) mutants were sev...

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Bibliographic Details
Published in:Physiology & behavior 2005-09, Vol.86 (1-2), p.46-51
Main Authors: LALONDE, R, MARCHETTI, N, STRAZIELLE, C
Format: Article
Language:English
Subjects:
Analysis of Variance
Animals
Behavior, Animal - physiology
Biological and medical sciences
Body Weight
Dystonia Musculorum Deformans - genetics
Dystonia Musculorum Deformans - physiopathology
Exploratory Behavior - physiology
Female
Fundamental and applied biological sciences. Psychology
Male
Mice
Mice, Neurologic Mutants
Motor Activity - genetics
Motor control and motor pathways. Reflexes. Control centers of vegetative functions. Vestibular system and equilibration
Neurologic Examination
Posture - physiology
Psychomotor Performance - physiology
Reaction Time - physiology
Reflex - physiology
Spinocerebellar Ataxias - genetics
Spinocerebellar Ataxias - physiopathology
Statistics, Nonparametric
Vertebrates: nervous system and sense organs
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Summary:The autosomal recessive dystonia musculorum (Dst(dt-J)) mutation causes degenerative lesions of peripheral and central sensory pathways. A test battery of motor, sensory, postural, and autonomic functions was used to compare young control and homozygous Dst(dt-J) mice. The Dst(dt-J) mutants were severely impaired for muscle strength, limb coordination, and postural reflexes. As a result of a loss in motor control, the mutants were hypoactive in the open-field and fell quickly from the stationary beam. In sensory tests, the acoustic startle response was impaired, but not tactile reflexes and contact righting, attesting to preserved labyrinthine function and non-lemniscal pathways. Dst(dt-J) mutants were also distinguishable from controls on the basis of tremor, a paler skin, piloerection, and half-open eyes, as well as low body weight and fecal boli. Grooming episodes were less frequent in the mutants but without any reduction in grooming time. The neurologic screening battery delineated the functional integrity of some sensorimotor pathways in a spinocerebellar mutant whose severe phenotype prevents a more elaborate evaluation.
ISSN:0031-9384
1873-507X
DOI:10.1016/j.physbeh.2005.06.028