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Alexander disease
Alexander disease, also known as fibrinoid leukodystrophy, is a progressive and fatal neurodegenerative disease. It is a rare genetic disorder and mostly affects infants and children, causing developmental delay and changes in physical characteristics.〔("MUTATION KEY TO ALEXANDER DISEASE" - United Press International )〕〔(GeneReviews/NCBI/NIH/UW entry on Alexander disease )〕〔
==Cause==
Alexander disease is a genetic disorder affecting the midbrain and cerebellum of the central nervous system. It is caused by mutations in the gene for glial fibrillary acidic protein (GFAP) that maps to chromosome 17q21. It is inherited in an autosomal dominant manner, such that the child of a parent with the disease has a 50% chance of inheriting the condition, if the parent is heterozygotic. However, most cases arise ''de novo'' as the result of sporadic mutations.〔
Alexander disease belongs to leukodystrophies, a group of diseases that affect the growth or development of the myelin sheath. The destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers.〔〔("Cause of brain disease found" -BBC News )〕Rosenthal fibers appear not to be present in healthy people,〔〔http://www.ulf.org/types/Alexander.html〕 but occur in specific diseases, like some forms of cancer.〔〔 The Rosenthal fibers found in Alexander disease do not share the distribution or concentration of other diseases and disorders.〔
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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