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Muscle Nerve ; 57(5): 814-820, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29193204

RESUMEN

INTRODUCTION: Agrin is essential for the formation and maintenance of neuromuscular junctions (NMJs). NT-1654 is a C-terminal fragment of mouse neural agrin. In this study, we determined the effects of NT-1654 on the severity of experimental autoimmune myasthenia gravis (EAMG). METHODS: EAMG was induced in female Lewis rats by immunization with the Torpedo acetylcholine receptor (tAChR) and complete Freund's adjuvant (CFA). NT-1654 was dissolved in phosphate-buffered saline (PBS) and injected daily subcutaneously into tAChR immunized rats during the first 10 days after immunization, and then every other day for the following 20 days. RESULTS: We showed that NT-1654 attenuated clinical severity, effectively promoted the clustering of AChRs at NMJs, and alleviated the impairment of NMJ transmission and the reduction of muscle-specific kinase (MuSK) in EAMG rats. DISCUSSION: We demonstrated that NT-1654 attenuated clinical severity, effectively promoted the clustering of AChRs at NMJs, and alleviated the impairment of NMJ transmission and the reduction of muscle-specific kinase (MuSK) in EAMG rats. Muscle Nerve 57: 814-820, 2018.


Asunto(s)
Agrina/uso terapéutico , Inmunización/efectos adversos , Miastenia Gravis Autoinmune Experimental/tratamiento farmacológico , Miastenia Gravis Autoinmune Experimental/patología , Fragmentos de Péptidos/uso terapéutico , Potenciales de Acción/fisiología , Agrina/biosíntesis , Agrina/química , Animales , Autoanticuerpos/metabolismo , Modelos Animales de Enfermedad , Relación Dosis-Respuesta a Droga , Electromiografía , Femenino , Adyuvante de Freund/toxicidad , Regulación de la Expresión Génica/efectos de los fármacos , Glicoproteínas de Membrana/metabolismo , Músculo Esquelético/patología , Atrofia Muscular/etiología , Atrofia Muscular/terapia , Proteínas del Tejido Nervioso/metabolismo , Neurofibromina 1/metabolismo , Unión Neuromuscular/patología , Fragmentos de Péptidos/biosíntesis , Fragmentos de Péptidos/química , Ratas , Ratas Endogámicas Lew , Receptores Colinérgicos/inmunología , Receptores Colinérgicos/metabolismo
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