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Publication : Neurobehavioral performances and brain regional metabolism in Dab1(scm) (scrambler) mutant mice.

First Author  Jacquelin C Year  2013
Journal  Behav Brain Res Volume  252
Pages  92-100 PubMed ID  23707934
Mgi Jnum  J:198993 Mgi Id  MGI:5500091
Doi  10.1016/j.bbr.2013.05.024 Citation  Jacquelin C, et al. (2013) Neurobehavioral performances and brain regional metabolism in Dab1 (scrambler) mutant mice. Behav Brain Res 252C:92-100
abstractText  As disabled-1 (DAB1) protein acts downstream in the reelin signaling pathway modulating neuronal migration, glutamate neurotransmission, and cytoskeletal function, the disabled-1 gene mutation (scrambler or Dab1scm mutation) results in ataxic mice displaying dramatic neuroanatomical defects similar to those observed in the reeler gene (Reln) mutation. By comparison to non-ataxic controls, Dab1scm mutants showed severe motor coordination impairments on stationary beam, coat-hanger, and rotorod tests but were more active in the open-field. Dab1scm mutants were also less anxious in the elevated plus-maze but with higher latencies in the emergence test. In mutants versus controls, changes in regional brain metabolism as measured by cytochrome oxidase (COX) activity occurred mainly in structures intimately connected with the cerebellum, in basal ganglia, in limbic regions, particularly hippocampus, as well as in visual and parietal sensory cortices. Although behavioral results characterized a major cerebellar disorder in the Dab1scm mutants, motor activity impairments in the open-field were associated with COX activity changes in efferent basal ganglia structures such as the substantia nigra, pars reticulata. Metabolic changes in this structure were also associated with the anxiety changes observed in the elevated plus-maze and emergence test. These results indicate a crucial participation of the basal ganglia in the functional phenotype of ataxic Dab1scm mutants.
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