Preferred Name

Alexander Disease

Synonyms
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).

ID

http://uri.neuinfo.org/nif/nifstd/birnlex_12528

createdDate

2007-10-05

definingCitationURI

http://www.ninds.nih.gov/disorders/alexander_disease/alexander_disease.htm

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).

hasBirnlexCurator

http://uri.neuinfo.org/nif/nifstd/readable/Bill_Bug

hasCurationStatus

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasExternalSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH

label

Alexander Disease

MeshUid

D038261

modifiedDate

April 11, 2009

preferred label

Alexander Disease

synonym

Alexander's Disease

Alexanders Disease

subClassOf

http://uri.neuinfo.org/nif/nifstd/birnlex_12526

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