Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Más filtros












Base de datos
Intervalo de año de publicación
1.
Genes Brain Behav ; 18(8): e12607, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31437340

RESUMEN

Multiple clinical and experimental evidences suggest that amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are members of a disease continuum. Pathological inclusions of fused in sarcoma (FUS) protein have been observed in subsets of patients with these diseases but their anatomical distribution is different for two diseases. These structures are present in motor neurons in ALS cases but in cortical neurons in FTLD cases. Expression of a C-terminally truncated form of human FUS causes an early onset and progressive motor neuron pathology in transgenic mice but only when these neurons express a certain level of this protein. Severe motor dysfunction and early lethality of mice with expression above this level prevent their use for studies of FTLD-related pathology caused by expression of this form of FUS. In the present study, we used another line of mice expressing the same protein but not developing any signs of motor system dysfunction due to substantially lower level of transgene expression in motor neurons. In a set of tests 5-month old mice displayed certain behavioural abnormalities, including increased impulsivity, decreased anxiety and compromised social interaction, which recapitulate behaviour characteristics typically seen in FTLD patients.


Asunto(s)
Conducta Animal , Demencia Frontotemporal/genética , Proteína FUS de Unión a ARN/genética , Animales , Condicionamiento Clásico , Lóbulo Frontal/metabolismo , Lóbulo Frontal/patología , Humanos , Masculino , Ratones , Ratones Endogámicos C57BL , Neuronas Motoras/metabolismo , Movimiento , Conducta Social , Transgenes
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...