Ribonuclease H2 Subunit A (RNASEH2A), Polyclonal Antibody

Rnase-H2A; RNASEHI; RNHIA; RNHL; AGS4; Ribonuclease H2,Large Subunit; Aicardi-Goutieres Syndrome 4; Ribonuclease HI large subunit

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-RNASEH2A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC)

33,395 Da

ribonuclease H2 subunit A

ribonuclease H2 subunit A

Ribonuclease H2 subunit A

RNASEHI; RNHIA; RNase H2 subunit A; AGS4; RNase HI large subunit  [Similar Products]

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 ribonucleotides. It is predicted to remove Okazaki fragment RNA primers during lagging strand DNA synthesis and to excise single ribonucleotides from DNA-DNA duplexes. Mutations in this gene cause Aicardi-Goutieres Syndrome (AGS), a 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.[provided by RefSeq, Aug 2009]

RNASEH2A: Catalytic subunit of RNase HII, an endonuclease that specifically degrades the RNA of RNA:DNA hybrids. Participates in DNA replication, possibly by mediating the removal of lagging- strand Okazaki fragment RNA primers during DNA replication. Mediates the excision of single ribonucleotides from DNA:RNA duplexes. Defects in RNASEH2A are the cause of Aicardi-Goutieres syndrome type 4 (AGS4). A form of Aicardi-Goutieres syndrome, a genetically heterogeneous disease characterized by cerebral atrophy, leukoencephalopathy, intracranial calcifications, chronic cerebrospinal fluid (CSF) lymphocytosis, increased CSF alpha-interferon, and negative serologic investigations for common prenatal infection. Clinical features as thrombocytopenia, hepatosplenomegaly and elevated hepatic transaminases along with intermittent fever may erroneously suggest an infective process. Severe neurological dysfunctions manifest in infancy as progressive microcephaly, spasticity, dystonic posturing and profound psychomotor retardation. Death often occurs in early childhood. Belongs to the RNase HII family. Eukaryotic subfamily.

Protein type: DNA replication; EC 3.1.26.4; Ribonuclease

Chromosomal Location of Human Ortholog: 19p13.13

Cellular Component: cytosol; nucleoplasm

Molecular Function: metal ion binding; ribonuclease activity; ribonuclease H activity; RNA binding

Biological Process: DNA replication; DNA replication, removal of RNA primer; mismatch repair; RNA catabolic process

Disease: Aicardi-goutieres Syndrome 4

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