[HTML][HTML] The structure of the human RNase H2 complex defines key interaction interfaces relevant to enzyme function and human disease

MAM Reijns, D Bubeck, LCD Gibson… - Journal of Biological …, 2011 - ASBMB
Ribonuclease H2 (RNase H2) is the major nuclear enzyme involved in the degradation of
RNA/DNA hybrids and removal of ribonucleotides misincorporated in genomic DNA.
Mutations in each of the three RNase H2 subunits have been implicated in a human auto-
inflammatory disorder, Aicardi-Goutières Syndrome (AGS). To understand how mutations
impact on RNase H2 function we determined the crystal structure of the human heterotrimer.
In doing so, we correct several key regions of the previously reported murine RNase H2 …