Preferred Name |
Alexander Disease |
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Synonyms |
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Definitions |
A rare inherited disorder of myelin formation. Alexander disease is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of cytoplasmic inclusions called Rosenthal fibers. The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with ALPHA-CRYSTALLIN B CHAIN. Rosenthal fibers are found predominantly in ASTROCYTES located in the subependymal, subpial, and periventricular areas of the BRAIN (MeSH). |
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ID |
http://uri.neuinfo.org/nif/nifstd/birnlex_12528 |
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createdDate |
2007-10-05 |
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definingCitationURI |
http://www.ninds.nih.gov/disorders/alexander_disease/alexander_disease.htm |
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definition |
A rare inherited disorder of myelin formation. Alexander disease is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of cytoplasmic inclusions called Rosenthal fibers. The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with ALPHA-CRYSTALLIN B CHAIN. Rosenthal fibers are found predominantly in ASTROCYTES located in the subependymal, subpial, and periventricular areas of the BRAIN (MeSH). |
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hasBirnlexCurator | ||
hasCurationStatus | ||
hasExternalSource | ||
label |
Alexander Disease |
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MeshUid |
D038261 |
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modifiedDate |
April 11, 2009 |
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preferred label |
Alexander Disease |
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synonym |
Alexander's Disease Alexanders Disease |
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subClassOf |