disease:Defects in RANBP2 are the cause of susceptibility to acute necrotizing encephalopathy type 1 (ANE1) [MIM:608033]. Acute necrotizing encephalopathy (ANE) is a rapidly progressive encephalopathy, seizures, and coma that can occur within days in otherwise healthy children after common viral infections such as influenza and parainfluenza, without evidence of viral infection of the brain or inflammatory cell infiltration. Brain T2-weighted magnetic resonance imaging reveals characteritic symmetric lesions present in the thalami, pons, and brainstem.,domain:Contains F-X-F-G repeats.,function:E3 SUMO-protein ligase which facilitates SUMO1 and SUMO2 conjugation by UBE2I. Involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain which acts as a docking site for substrates. Could also have isomerase or chaperone activity and may bind R

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