RNASEH2A

Ribonuclease H2 subunit A, also known as RNase H2 subunit A, is an is_associated_with::enzyme that in humans is encoded by the RNASEH2A is_associated_with::gene.

Function
The protein encoded by this gene is a component of the heterotrimeric type II ribonuclease H enzyme (RNAseH2). RNAseH2 is the major source of ribonuclease H activity in mammalian cells and endonucleolytically cleaves is_associated_with::ribonucleotides. It is predicted to remove is_associated_with::Okazaki fragment RNA primers during lagging strand is_associated_with::DNA synthesis and to excise single ribonucleotides from DNA-DNA duplexes.

Clinical significance
Mutations in this gene cause is_associated_with::Aicardi-Goutieres syndrome (AGS), an autosomal recessive neurological disorder characterized by progressive is_associated_with::microcephaly and psychomotor retardation, intracranial calcifications, elevated levels of interferon-alpha and white blood cells in the is_associated_with::cerebrospinal fluid.