Ran Binding Protein 2 (
RanBP2 or Nucleoporin358) is one of the main components of the cytoplasmic filaments of the nuclear pore complex. Mutations in the
RANBP2 gene are associated with acute necrotizing
encephalopathy type 1 (ANE1), a rare condition where patients experience a sharp rise in
cytokine production in response to
viral infection and undergo hyperinflammation,
seizures,
coma, and a high rate of mortality. Despite this, it remains unclear howRanBP2 and its ANE1-associated mutations contribute to pathology. Mounting evidence has shown that
RanBP2 interacts with distinct viruses to regulate
viral infection. In addition,
RanBP2 may regulate innate immune response pathways. This review summarizes recent advances in our understanding of how mutations in
RANBP2 contribute to ANE1 and discusses how
RanBP2 interacts with distinct viruses and affects
viral infection. Recent findings indicate that
RanBP2 might be an important therapeutic target, not only in the suppression of ANE1-driven
cytokine storms, but also to combat hyperinflammation in response to
viral infections.