ribonuclease H2 subunit A (RNase H2 subunit A, ribonuclease HI subunit A, ribonuclease HI large subunit, RNase HI large subunit, RNase H35, Aicardi-Goutieres syndrome 4 protein, AGS4, RNASEH2A, RNASEHI, RNHIA)

Function

• catalytic subunit of RNase H2

Cofactor:

• Mn+2 or Mg+2
• binds 1 divalent metal ion per monomer in absence of substrate
• may bind a 2nd metal ion after substrate binding

Compartment

nucleus

Pathology

• defects in RNASEH2A result in Aicardi-Goutieres syndrome 4

More general terms

• enzyme catalytic subunit

Component of

• ribonuclease H2 (RNase H2)

Database

• OMIM: https://mirror.omim.org/entry/606034
• UniProt: http://www.uniprot.org/uniprot/O75792.html