IGF-1 in Friedreich’s Ataxia – proof-of-concept trial

[Background] Friedreich’s ataxia is an autosomal recessive, severely incapacitating disorder. There is little objective evidence regarding FRDA management. Abnormalities in the insulin/insulin-like growth factor 1 (IGF-1) system (IIS) signalling pathway were thought to play a role in the physiopatho...

Descripción completa

Detalles Bibliográficos
Autores: Sanz-Gallego, Irene, Torres Alemán, Ignacio, Arpa, Javier
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2014
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/125781
Acceso en línea:http://hdl.handle.net/10261/125781
Access Level:acceso abierto
Palabra clave:Friedreich’s ataxia
IGF-1 therapy
Descripción
Sumario:[Background] Friedreich’s ataxia is an autosomal recessive, severely incapacitating disorder. There is little objective evidence regarding FRDA management. Abnormalities in the insulin/insulin-like growth factor 1 (IGF-1) system (IIS) signalling pathway were thought to play a role in the physiopathological processes of various neurodegenerative disorders, including spinocerebellar ataxias. The objective of the study was to test the safety, tolerability, and efficacy of therapy with IGF-1 in Friedreich’s ataxia (FRDA) patients in a clinical pilot study.