Loss of motoneuron-specific microRNA-218 causes systemic neuromuscular failure.
Science
; 350(6267): 1525-9, 2015 Dec 18.
Article
en En
| MEDLINE
| ID: mdl-26680198
ABSTRACT
Dysfunction of microRNA (miRNA) metabolism is thought to underlie diseases affecting motoneurons. One miRNA, miR-218, is abundantly and selectively expressed by developing and mature motoneurons. Here we show that mutant mice lacking miR-218 die neonatally and exhibit neuromuscular junction defects, motoneuron hyperexcitability, and progressive motoneuron cell loss, all of which are hallmarks of motoneuron diseases such as amyotrophic lateral sclerosis and spinal muscular atrophy. Gene profiling reveals that miR-218 modestly represses a cohort of hundreds of genes that are neuronally enriched but are not specific to a single neuron subpopulation. Thus, the set of messenger RNAs targeted by miR-218, designated TARGET(218), defines a neuronal gene network that is selectively tuned down in motoneurons to prevent neuromuscular failure and neurodegeneration.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Regulación de la Expresión Génica
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Enfermedad de la Neurona Motora
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Enfermedades Neurodegenerativas
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MicroARNs
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Neuronas Motoras
Tipo de estudio:
Etiology_studies
Límite:
Animals
Idioma:
En
Revista:
Science
Año:
2015
Tipo del documento:
Article