Cree encephalitis is allelic with Aicardi-Goutieres syndrome: implications for the pathogenesis of disorders of interferon alpha metabolism.
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ABSTRACT:
SUBMITTER: Crow YJ
PROVIDER: S-EPMC1735395 | biostudies-other | 2003 Mar
REPOSITORIES: biostudies-other
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Cree encephalitis is allelic with Aicardi-Goutiéres syndrome: implications for the pathogenesis of disorders of interferon alpha metabolism.
Crow Y J YJ Black D N DN Ali M M Bond J J Jackson A P AP Lefson M M Michaud J J Roberts E E Stephenson J B P JB Woods C G CG Lebon P P
Journal of medical genetics 20030301 3
Aicardi-Goutiéres syndrome (AGS) is an early onset, progressive encephalopathy characterised by calcification of the basal ganglia, white matter abnormalities, and a chronic cerebrospinal fluid (CSF) lymphocytosis. Cree encephalitis shows phenotypic overlap with AGS although the conditions have been considered distinct because of immunological abnormalities observed in Cree encephalitis. We report that levels of interferon alpha (IFN-alpha), a marker of AGS, are raised in Cree encephalitis. More ...[more]
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