[HTML][HTML] The structural and biochemical characterization of human RNase H2 complex reveals the molecular basis for substrate recognition and Aicardi-Goutières …

M Figiel, H Chon, SM Cerritelli, M Cybulska… - Journal of Biological …, 2011 - ASBMB
RNase H2 cleaves RNA sequences that are part of RNA/DNA hybrids or that are
incorporated into DNA, thus, preventing genomic instability and the accumulation of aberrant
nucleic acid, which in humans induces Aicardi-Goutières syndrome, a severe autoimmune
disorder. The 3.1 Å crystal structure of human RNase H2 presented here allowed us to map
the positions of all 29 mutations found in Aicardi-Goutières syndrome patients, several of
which were not visible in the previously reported mouse RNase H2. We propose the …