[HTML][HTML] Z-DNA and Z-RNA in human disease

A Herbert - Communications biology, 2019 - nature.com
Abstract 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 …

[HTML][HTML] Adenosine-to-inosine editing of endogenous Z-form RNA by the deaminase ADAR1 prevents spontaneous MAVS-dependent type I interferon responses

Q Tang, RE Rigby, GR Young, AK Hvidt, T Davis… - Immunity, 2021 - cell.com
Nucleic acids are powerful triggers of innate immunity and can adopt the Z-conformation, an
unusual left-handed double helix. Here, we studied the biological function (s) of Z-RNA …

The crystal structure of the Zβ domain of the RNA-editing enzyme ADAR1 reveals distinct conserved surfaces among Z-domains

A Athanasiadis, D Placido, S Maas, BA Brown II… - Journal of molecular …, 2005 - Elsevier
The Zα domains represent a growing subfamily of the winged helix-turn-helix (HTH) domain
family whose members share a remarkable ability to bind specifically to Z-DNA and/or Z …

Mendelian disease caused by variants affecting recognition of Z-DNA and Z-RNA by the Zα domain of the double-stranded RNA editing enzyme ADAR

A Herbert - European Journal of Human Genetics, 2020 - nature.com
Variants in the human double-stranded RNA editing enzyme ADAR produce three well-
characterized rare Mendelian Diseases: Dyschromatosis Symmetrica Hereditaria (OMIM …

[HTML][HTML] Recognition of non-CpG repeats in Alu and ribosomal RNAs by the Z-RNA binding domain of ADAR1 induces AZ junctions

PJ Nichols, S Bevers, M Henen, JS Kieft… - Nature …, 2021 - nature.com
Abstract Adenosine-to-inosine (A-to-I) editing of eukaryotic cellular RNAs is essential for
protection against auto-immune disorders. Editing is carried out by ADAR1, whose innate …

[HTML][HTML] ADAR1 interaction with Z-RNA promotes editing of endogenous double-stranded RNA and prevents MDA5-dependent immune activation

R de Reuver, E Dierick, B Wiernicki, K Staes, L Seys… - Cell reports, 2021 - cell.com
Loss of function of adenosine deaminase acting on double-stranded RNA (dsRNA)-1
(ADAR1) causes the severe autoinflammatory disease Aicardi-Goutières syndrome (AGS) …

[HTML][HTML] To “Z” or not to “Z”: Z-RNA, self-recognition, and the MDA5 helicase

A Herbert - PLoS Genetics, 2021 - journals.plos.org
Double-stranded RNA (dsRNA) is produced both by virus and host. Its recognition by the
melanoma differentiation–associated gene 5 (MDA5) initiates type I interferon responses …

[HTML][HTML] A left-handed RNA double helix bound by the Zα domain of the RNA-editing enzyme ADAR1

D Placido, BA Brown, K Lowenhaupt, A Rich… - Structure, 2007 - cell.com
The A form RNA double helix can be transformed to a left-handed helix, called Z-RNA.
Currently, little is known about the detailed structural features of Z-RNA or its involvement in …

[HTML][HTML] ADAR1 averts fatal type I interferon induction by ZBP1

H Jiao, L Wachsmuth, S Wolf, J Lohmann, M Nagata… - Nature, 2022 - nature.com
Mutations of the ADAR1 gene encoding an RNA deaminase cause severe diseases
associated with chronic activation of type I interferon (IFN) responses, including Aicardi …

Left-handed Z-DNA: structure and function

A Herbert, A Rich - Genetica, 1999 - Springer
Z-DNA is a high energy conformer of B-DNA that forms in vivo during transcription as a result
of torsional strain generated by a moving polymerase. An understanding of the biological …