RNAse H2A, Polyclonal Antibody

RNAse H2A; AGS4; JUNB; RNHL; RNHIA; RNASEHI; Ribonuclease H2 subunit A; Aicardi-Goutieres syndrome 4 protein; RNase H2 subunit A; ribonuclease H2, subunit A

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

Polyclonal

IgG

Rabbit

RNAse H2A Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

RNAse H2A antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

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.

RNAse H2A Antibody: Ribonucleases (RNAses) H are enzymes that hydrolyze the RNA strands of RNA/DNA hybrids. The major role of these enzymes is to remove the RNA strand from the RNA/DNA hybrids that form during DNA replication and repair. RNAse H2 is made up of three subunits; all three are required for RNAse activity. Recent evidence has demonstrated that mutations in RNAse H2A or any of the other subunits result in Aicardi-Goutieres syndrome (AGS), a neurological disorder with similar symptoms to viral brain infections including high levels of IFN-alpha in the cerebral spinal fluid. Similar conditions are observed with mutations in TREX1, a single-stranded DNA exonuclease, suggesting that RNAse H2 and TREX1 may have similar roles, and that mutations in any of these genes lead to an accumulation of intracellular nucleic acids, triggering an inflammatory response through activation of the innate immune system.

ELISA (EIA), Western Blot (WB), Immunocytochemistry (ICC), Immunofluorescence (IF)

RNAse H2A antibody can be used for detection of RNAse H2A by Western blot at 1 mug/mL. Antibody can also be used for immunocytochemistry starting at 2 mug/mL. For immunofluorescence start at 4 mug/mL.

Western blot analysis of RNAse H2A in HeLa cell lysate with RNAse H2A antibody at 1 μg/mL.

Immunocytochemistry of RNAse H2A in HeLa cells with RNAse H2A antibody at 2 μg/mL.

Immunofluorescence of RNase H2A in Hela cells with RNAse H2A antibody at 5 μg/mL.

33,395 Da

ribonuclease H2, subunit A

ribonuclease H2 subunit A; RNase H(35); RNase H2 subunit A; RNase HI large subunit; ribonuclease HI subunit A; ribonuclease HI large subunit; ribonuclease H2, large subunit; ribonuclease HI, large subunit; aicardi-Goutieres syndrome 4 protein

Ribonuclease H2 subunit A

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

RNH2A_HUMAN

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: Ribonuclease; EC 3.1.26.4; DNA replication

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: nucleus

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

Biological Process: mismatch repair; RNA catabolic process; DNA replication

Disease: Aicardi-goutieres Syndrome 4

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