Premium
P2–011: Transmissibility of prion diseases in a lemurian primate, Microcebus murinus
Author(s) -
Mestre-Frances Nadine,
Crozet Carole,
Lehmann Sylvain,
Rouland Sylvie,
Perret-Liaudet Arm,
Verdier Jean-Michel
Publication year - 2006
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2006.05.848
Subject(s) - gliosis , spongiosis , thalamus , basal ganglia , myoclonus , pathology , scrapie , brainstem , creutzfeldt jakob syndrome , neuropil , neuroscience , medicine , virology , biology , disease , central nervous system , prion protein
[1999] 274, 2243). We reported the cellular functions ( J. Biol. Chem. [2000] 275, 13056; J. Biol. Chem. [2003] 278, 49448; J. Biol. Chem. [2004] 279, 21628; J. Biol. Chem. [2004] 279, 24343). To analyze the physiological function of X11L and X11 in vivo is important for understanding comprehensive neural activity and the mechanism of neurodegenerative disease. Previous analysis reported that X11 gene-deficient [X11-/-] mice showed no defects in excitatory synaptic transmission and only a subtle defect in dopamine and GABA transmission (Neurosci. Res. [2002] 43, 251, Proc. Natl. Acad. Sci. USA [2003] 100, 1409). Here we generate X11L gene-deficient [X11L-/-] mice and X11/X11L gene-deficient [X11-/-, X11L-/-] mice and analyze their phenotypes. Methods: Genotype analysis of offspring and survival rate of each genotype were examined. We monitored behavior of [X11-/-, X11L] mice and examined presence or absence of abnormal behavior. Brain morphology was explored by producing brain tissue-sections, which were analyzed by chemicaland immuno-staining. Results: We found that each of the genotype mice are viable and fertile. Both [X11-/-] and [X11L-/-] mice do not show remarkable behavioral and brain morphological abnormality, while [X11-/-, X11L-/-] mice showed obvious abnormality in behavior and neural activity. After this abnormal behavior, we observed alternation of c-Fos immunoreactivity in several regions. Conclusions: Our observations suggest that X11 and X11L play complementary in part to preserve neural function and that deficiency of both genes induces crisis of neural function, leading to neuropathology.