Details of Disease | DrugMAP

General Information of Disease (ID: DISPCVGG)

Disease Name	Familial acute necrotizing encephalopathy
Synonyms	acute necrotizing encephalopathy type 1; ANE1; susceptibility to acute necrotizing encephalopathy; encephalopathy, acute, infection-induced, susceptibility to, 3; susceptibility to infection-induced acute encephalopathy 3; susceptibility to acute infection-induced encephalopathy-3; Postinfectious acute necrotizing hemorrhagic encephalopathy; encephalopathy, acute necrotizing, susceptibility to; autosomal dominant acute necrotizing encephalopathy; IIAE3; infection-induced acute encephalopathy 3; ADANE; encephalopathy, acute, infection-induced, susceptibility to, type 3; encephalopathy, acute, infection-induced, 3, susceptibility to; recurrent acute necrotizing encephalopathy
Definition	Familial acute necrotizing encephalopathy or ADANE is a potentially fatal neurological disease characterized by neuropathological lesions principally involving the brainstem, thalamus and putamen.
Disease Hierarchy	DISRRNDL: Encephalopathy, acute, infection-induced DISP9UN3: Mendelian encephalopathy DISPCVGG: Familial acute necrotizing encephalopathy
Disease Identifiers	MONDO ID MONDO_0011953 UMLS CUI C2675556 OMIM ID 608033 MedGen ID 382634 Orphanet ID 88619

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 1 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
RANBP2	OTFG5CVF	Strong	Autosomal dominant	[1]
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References

1	Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. Am J Hum Genet. 2009 Jan;84(1):44-51. doi: 10.1016/j.ajhg.2008.12.009.