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RNASEH2A
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3P56, 3PUF
Identifiers
Aliases	RNASEH2A, AGS4, JUNB, RNASEHI, RNHIA, RNHL, ribonuclease H2 subunit A, THSD8
External IDs	OMIM: 606034; MGI: 1916974; HomoloGene: 4664; GeneCards: RNASEH2A; OMA:RNASEH2A - orthologs
Gene location (Human)
Chr.	Chromosome 19 (human)
End	12,813,640 bp
Gene location (Mouse)
Chr.	Chromosome 8 (mouse)
End	85,696,396 bp
RNA expression pattern
	Top expressed in
	ganglionic eminence; ; ventricular zone; ; gonad; ; mucosa of transverse colon; ; granulocyte; ; stromal cell of endometrium; ; placenta; ; lymph node; ; mucosa of esophagus; ; bone marrow;
	Top expressed in
	seminiferous tubule; ; spermatid; ; spermatocyte; ; gastrula; ; ventricular zone; ; embryo; ; embryo; ; hand; ; epiblast; ; otic vesicle;
	More reference expression data
	n/a
Gene ontology
Molecular function	RNA binding; nucleic acid binding; nuclease activity; endonuclease activity; RNA-DNA hybrid ribonuclease activity; ribonuclease activity; hydrolase activity; metal ion binding;
Cellular component	ribonuclease H2 complex; nucleus; nucleoplasm; cytosol;
Biological process	DNA mismatch repair; DNA replication; RNA catabolic process; RNA metabolic process; nucleic acid phosphodiester bond hydrolysis; RNA phosphodiester bond hydrolysis, endonucleolytic; RNA phosphodiester bond hydrolysis; DNA replication, removal of RNA primer;
	Sources:Amigo / QuickGO
Orthologs
	10535
	69724
	ENSG00000104889
	ENSMUSG00000052926
	O75792
	Q9CWY8
	NM_006397
	NM_027187; NM_001364370
	NP_006388
	NP_081463; NP_001351299
	Wikidata
View/Edit Human	View/Edit Mouse

Ribonuclease H2 subunit A, also known as RNase H2 subunit A, is an enzyme that in humans is encoded by the RNASEH2A gene.^[5]

Function

The protein encoded by this gene is a component of the heterotrimeric type II ribonuclease H enzyme (RNaseH2). The other two subunits are the non-catalytic RNASEH2B and RNASEH2C. RNaseH2 is the major source of ribonuclease H activity in mammalian cells and endonucleolytically cleaves ribonucleotides. It is predicted to remove Okazaki fragment RNA primers during lagging strand DNA synthesis and to excise single ribonucleotides from DNA-DNA duplexes.^[5]

Clinical significance

Mutations in this gene cause Aicardi–Goutières syndrome (AGS), an autosomal recessive neurological disorder characterized by progressive microcephaly and psychomotor retardation, intracranial calcifications, elevated levels of interferon-alpha and white blood cells in the cerebrospinal fluid.^[5]