Article
Murine model of Alexander disease: analysis of GFAP aggregate formation and its pathological significance.
Glia - 15 Apr 2007
Tanaka Kenji F, Takebayashi Hirohide, Yamazaki Yoshihiko, Ono Katsuhiko, Naruse Masae, Iwasato Takuji, Itohara Shigeyoshi, Kato Hiroshi, Ikenaka Kazuhiro
Abstract excerpt
Alexander disease is caused by a coding mutation in the glial fibrillary acidic protein (GFAP) gene. The pathological hallmark is the formation of cytoplasmic inclusions within astrocytes known as Rosenthal fibers (RFs), which primarily consist of GFAP and several heat shock proteins. The presence of mutant GFAP would appear to be involved in RF formation; however, overproduction of wild type human GFAP in mouse...
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