Open AccessZ-DNA and Z-RNA in human disease
Author(s) -
Alan Herbert
Publication year - 2019
Publication title -
communications biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.812
H-Index - 26
ISSN - 2399-3642
DOI - 10.1038/s42003-018-0237-x
Subject(s) - adar , retrotransposon , rna , biology , innate immune system , rna editing , alu element , dna , genetics , genome , rna silencing , intron , human genome , rna interference , gene , immune system , transposable element
Left-handed Z-DNA/Z-RNA is bound with high affinity by the Zα domain protein family that includes ADAR (a double-stranded RNA editing enzyme), ZBP1 and viral orthologs regulating innate immunity. Loss-of-function mutations in ADAR p150 allow persistent activation of the interferon system by Alu dsRNAs and are causal for Aicardi-Goutières Syndrome. Heterodimers of ADAR and DICER1 regulate the switch from RNA- to protein-centric immunity. Loss of DICER1 function produces age-related macular degeneration, a different type of Alu-mediated disease. The overlap of Z-forming sites with those for the signal recognition particle likely limits invasion of primate genomes by Alu retrotransposons.