Metabolic and behavioral effects of mutant huntingtin deletion in Sim1 neurons in the BACHD mouse model of Huntington's disease.

摘要: Hypothalamic pathology, metabolic dysfunction and psychiatric symptoms are part of Huntington disease (HD), which is caused by an expanded CAG repeat in the huntingtin (HTT) gene. Inactivation mutant HTT selectively hypothalamus prevents development depressive-like behavior BACHD mouse model. The hypothalamic paraventricular nucleus (PVN) implicated emotional control, therefore we here tested whether inactivation PVN affects manifestations HD mice. mice were crossed with expressing Cre-recombinase under Sim1 promoter (Sim1-Cre) to inactivate cells, i.e. hypothalamus. We found that cells had a sex-specific effect on both phenotype, as these phenotypes no longer different male BACHD/Sim1-Cre compared wild-type littermates. also reduced number GnRH neurons specifically anterior increased testes weight Taken together, expression may play role for