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The RNase HII complex is an endonuclease that degrades RNA found in RNA:DNA duplexes and is composed of one catalytic subunit and two non-catalytic subunits. RNase HII-A, also called RNASEH2A (Ribonuclease H2 subunit A), RNASEHI, AGS4 or RNHIA, is the 299 amino acid catalytic subunit of RNase HII. Localized to the nucleus, RNase HII-A mediates the removal of Okazaki fragment RNA primers that are present on the lagging strand during DNA replication. RNase HII-A catalyzes the endonucleolytic cleavage of RNA to a 5'-phosphomonoester and is able to bind magnesium or manganese as cofactors. Defects in the gene encoding RNase HII-A are the cause of Aicardi-Goutieres syndrome type 4 (AGS4), an autosomal recessive encephalopathy characterized by cerebral atrophy, leukodystrophy, intracranial calcifications and chronic cerebrospinal fluid (CSF) lymphocytosis. Patients affected by AGS4 have severe neurological dysfunctions and often die in early childhood.
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps RNase HII-A (S-14LJ-17) | sc-101112 | 100 µg/ml | $333.00 |