Neuroprotective effects of granulocyte‐colony stimulating factor in a novel transgenic mouse model of SCA17

• DOI: 10.1111/j.1471-4159.2011.07304.x
• Publication Date: 2011-05-10T11:26:02Z
• Authors: Ya‐Chin Chang; Cheng‐Yueh Lin; Chen‐Ming Hsu; Hsin‐Chieh Lin; Yu‐Hsiang Chen; Guey‐Jen Lee‐Chen; Ming‐Tsan Su; Long‐Sun Ro; Chiung‐Mei Chen; Hsiu Mei Hsieh‐Li
• Keywords: Gliosis

Spinocerebellar ataxia type 17 (SCA17) is an autosomal dominant inherited disorder characterized by degeneration of spinocerebellar tracts and selected brainstem neurons owing to the expansion a CAG repeat human TATA-binding protein (hTBP) gene. To gain insight into pathogenesis this hTBP mutation, we generated transgenic mice with mutant gene driven Purkinje specific (Pcp2/L7) promoter. Mice expanded allele developed within 2-5 months. Behavioral analysis L7-hTBP showed reduced fall latency in rotarod assay. cell was identified immunostaining calbindin IP3R1. Reactive gliosis neuroinflammation occurred cerebellum, accompanied up-regulation GFAP Iba1. The were thus confirmed recapitulate SCA17 phenotype used as disease model explore potential granulocyte-colony stimulating factor treatment. Our results suggest that has neuroprotective effect these mice, ameliorating their neurological behavioral deficits. These data indicate expression cells sufficient produce phenotype, constitutes good for better neurodegeneration SCA17.