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NameRibonuclease H2 subunit A
Synonyms
  • 3.1.26.4
  • AGS4
  • Aicardi-Goutieres syndrome 4 protein
  • Ribonuclease HI large subunit
  • Ribonuclease HI subunit A
  • RNase H(35)
  • RNase H2 subunit A
  • RNase HI large subunit
  • RNASEHI
  • RNHIA
Gene NameRNASEH2A
OrganismHuman
Amino acid sequence
>lcl|BSEQ0019731|Ribonuclease H2 subunit A
MDLSELERDNTGRCRLSSPVPAVCRKEPCVLGVDEAGRGPVLGPMVYAICYCPLPRLADL
EALKVADSKTLLESERERLFAKMEDTDFVGWALDVLSPNLISTSMLGRVKYNLNSLSHDT
ATGLIQYALDQGVNVTQVFVDTVGMPETYQARLQQSFPGIEVTVKAKADALYPVVSAASI
CAKVARDQAVKKWQFVEKLQDLDTDYGSGYPNDPKTKAWLKEHVEPVFGFPQFVRFSWRT
AQTILEKEAEDVIWEDSASENQEGLRKITSYFLNEGSQARPRSSHRYFLERGLESATSL
Number of residues299
Molecular Weight33394.58
Theoretical pINot Available
GO Classification
Functions
  • RNA binding
  • metal ion binding
  • ribonuclease activity
  • RNA-DNA hybrid ribonuclease activity
Processes
  • DNA replication
  • mismatch repair
  • RNA catabolic process
  • RNA phosphodiester bond hydrolysis
  • RNA phosphodiester bond hydrolysis, endonucleolytic
  • DNA replication, removal of RNA primer
Components
  • cytoplasm
  • nucleoplasm
  • ribonuclease H2 complex
General FunctionRna-dna hybrid ribonuclease activity
Specific FunctionCatalytic 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.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDO75792
UniProtKB Entry NameRNH2A_HUMAN
Cellular LocationNucleus
Gene sequence
>lcl|BSEQ0019732|Ribonuclease H2 subunit A (RNASEH2A)
ATGGATCTCAGCGAGCTGGAGAGAGACAATACAGGCCGCTGTCGCCTGAGTTCGCCTGTG
CCCGCGGTGTGCCGCAAGGAGCCTTGCGTCCTGGGCGTCGATGAGGCGGGCAGGGGCCCC
GTGCTGGGCCCCATGGTCTACGCCATCTGTTATTGTCCCCTGCCTCGCCTGGCAGATCTG
GAGGCGCTGAAAGTGGCAGACTCAAAGACCCTATTGGAGAGCGAGCGGGAAAGGCTGTTT
GCGAAAATGGAGGACACGGACTTTGTCGGCTGGGCGCTGGATGTGCTGTCTCCAAACCTC
ATCTCTACCAGCATGCTTGGGCGGGTCAAATACAACCTGAACTCCCTGTCACATGATACA
GCCACTGGGCTTATACAGTATGCATTGGACCAGGGCGTGAACGTCACCCAGGTATTCGTG
GACACCGTAGGGATGCCAGAGACATACCAGGCGCGGCTGCAGCAAAGTTTTCCCGGGATT
GAGGTGACGGTCAAGGCCAAAGCAGATGCCCTCTACCCGGTGGTTAGTGCTGCCAGCATC
TGTGCCAAGGTGGCCCGGGACCAGGCCGTGAAGAAATGGCAGTTCGTGGAGAAACTGCAG
GACTTGGATACTGATTATGGCTCAGGCTACCCCAATGATCCCAAGACAAAAGCGTGGTTG
AAGGAGCACGTGGAGCCTGTGTTCGGCTTCCCCCAGTTTGTCCGGTTCAGCTGGCGCACG
GCCCAGACCATCCTGGAGAAAGAGGCGGAAGATGTTATATGGGAGGACTCAGCATCCGAG
AATCAGGAGGGACTCAGGAAGATCACATCCTACTTCCTCAATGAAGGGTCCCAAGCCCGT
CCCCGTTCTTCCCACCGATATTTCCTGGAACGCGGCCTGGAGTCAGCAACCAGCCTCTAG
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:18518
Chromosome Location19
LocusNot Available
References
  1. Frank P, Braunshofer-Reiter C, Wintersberger U, Grimm R, Busen W: Cloning of the cDNA encoding the large subunit of human RNase HI, a homologue of the prokaryotic RNase HII. Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):12872-7. 9789007
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  4. Dephoure N, Zhou C, Villen J, Beausoleil SA, Bakalarski CE, Elledge SJ, Gygi SP: A quantitative atlas of mitotic phosphorylation. Proc Natl Acad Sci U S A. 2008 Aug 5;105(31):10762-7. doi: 10.1073/pnas.0805139105. Epub 2008 Jul 31. 18669648
  5. Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. 21269460
  6. Bienvenut WV, Sumpton D, Martinez A, Lilla S, Espagne C, Meinnel T, Giglione C: Comparative large scale characterization of plant versus mammal proteins reveals similar and idiosyncratic N-alpha-acetylation features. Mol Cell Proteomics. 2012 Jun;11(6):M111.015131. doi: 10.1074/mcp.M111.015131. Epub 2012 Jan 5. 22223895
  7. Van Damme P, Lasa M, Polevoda B, Gazquez C, Elosegui-Artola A, Kim DS, De Juan-Pardo E, Demeyer K, Hole K, Larrea E, Timmerman E, Prieto J, Arnesen T, Sherman F, Gevaert K, Aldabe R: N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB. Proc Natl Acad Sci U S A. 2012 Jul 31;109(31):12449-54. doi: 10.1073/pnas.1210303109. Epub 2012 Jul 18. 22814378
  8. Figiel M, Chon H, Cerritelli SM, Cybulska M, Crouch RJ, Nowotny M: The structural and biochemical characterization of human RNase H2 complex reveals the molecular basis for substrate recognition and Aicardi-Goutieres syndrome defects. J Biol Chem. 2011 Mar 25;286(12):10540-50. doi: 10.1074/jbc.M110.181974. Epub 2010 Dec 22. 21177858
  9. Crow YJ, Leitch A, Hayward BE, Garner A, Parmar R, Griffith E, Ali M, Semple C, Aicardi J, Babul-Hirji R, Baumann C, Baxter P, Bertini E, Chandler KE, Chitayat D, Cau D, Dery C, Fazzi E, Goizet C, King MD, Klepper J, Lacombe D, Lanzi G, Lyall H, Martinez-Frias ML, Mathieu M, McKeown C, Monier A, Oade Y, Quarrell OW, Rittey CD, Rogers RC, Sanchis A, Stephenson JB, Tacke U, Till M, Tolmie JL, Tomlin P, Voit T, Weschke B, Woods CG, Lebon P, Bonthron DT, Ponting CP, Jackson AP: Mutations in genes encoding ribonuclease H2 subunits cause Aicardi-Goutieres syndrome and mimic congenital viral brain infection. Nat Genet. 2006 Aug;38(8):910-6. Epub 2006 Jul 16. 16845400
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  11. Ramantani G, Kohlhase J, Hertzberg C, Innes AM, Engel K, Hunger S, Borozdin W, Mah JK, Ungerath K, Walkenhorst H, Richardt HH, Buckard J, Bevot A, Siegel C, von Stulpnagel C, Ikonomidou C, Thomas K, Proud V, Niemann F, Wieczorek D, Hausler M, Niggemann P, Baltaci V, Conrad K, Lebon P, Lee-Kirsch MA: Expanding the phenotypic spectrum of lupus erythematosus in Aicardi-Goutieres syndrome. Arthritis Rheum. 2010 May;62(5):1469-77. doi: 10.1002/art.27367. 20131292