Dual transgenic reporter mice as a tool for monitoring expression of glial fibrillary acidic protein.

[alexander disease]

Glial fibrillary acidic protein (GFAP ) is the major intermediate filament protein of astrocytes , and its expression changes dramatically during development and following injury . To facilitate study of the regulation of GFAP expression , we have generated dual transgenic mice expressing both firefly luciferase under the control of a 2 .2 kb human GFAP promoter and Renilla luciferase under the control of a 0 .5 kb human Glyceraldehyde 3 phosphate dehydrogenase (GAPDH ) promoter for normalization of the GFAP signal . The GFAP -fLuc was highly expressed in brain compared to other tissues , and was limited to astrocytes , whereas the GAPDH -RLuc was more widely expressed . Normalization of the GFAP signal to the GAPDH signal reduced the inter-individual variability compared to using the GFAP signal alone . The GFAP /GAPDH ratio correctly reflected the up-regulation of GFAP that occurs following retinal degeneration in FVB /N mice because of the rd mutation . Following kainic acid-induced seizures , changes in the GFAP /GAPDH ratio precede those in total GFAP protein . In knock-in mice expressing the R 236 H Alexander disease mutant , GFAP promoter activity is only transiently elevated and may not entirely account for the accumulation of GFAP protein that takes place .

Diseases
Validation

Diseases presenting "major intermediate filament protein" symptom

alexander disease

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