LSM11, U7 small nuclear RNA associated
Last updated 26 Sept 2024
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The LSM11 gene provides instructions for making one piece (subunit) of a group of proteins called the U7 small nuclear ribonucleoprotein (snRNP) complex. In cells, the U7 snRNP complex plays an important role in processing molecules called messenger RNA (mRNA), which serve as the genetic blueprints for making proteins. The mRNA starts out as a molecule called pre-mRNA and must go through several processing steps before it is ready to make a protein. The U7 snRNP complex helps to process pre-mRNA. Specifically, the U7 snRNP complex targets the pre-mRNA of proteins called histones. Histones are structural proteins that attach (bind) to DNA and are necessary for copying a cell's DNA before the cell divides (a process known as DNA replication).
Normal function
The LSM11 gene provides instructions for making one piece (subunit) of a group of proteins called the U7 small nuclear ribonucleoprotein (snRNP) complex. In cells, the U7 snRNP complex plays an important role in processing molecules called messenger RNA (mRNA), which serve as the genetic blueprints for making proteins. The mRNA starts out as a molecule called pre-mRNA and must go through several processing steps before it is ready to make a protein. The U7 snRNP complex helps to process pre-mRNA. Specifically, the U7 snRNP complex targets the pre-mRNA of proteins called histones. Histones are structural proteins that attach (bind) to DNA and are necessary for copying a cell's DNA before the cell divides (a process known as DNA replication).
Conditions referring to this gene or chromosome
Other names
AGS8
FLJ38273
U7 SnRNA-Associated Sm-Like Protein LSm11
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Further reading and references
- LSM11, U7 small nuclear RNA associated; MedlinePlus, National Library of Medicine.
- Liu A, Ying S. Aicardi-Goutieres syndrome: A monogenic type I interferonopathy. Scand J Immunol. 2023 Oct;98(4):e13314. doi: 10.1111/sji.13314. Epub 2023 Jul 29.
- Naesens L, Nemegeer J, Roelens F, Vallaeys L, Meuwissen M, Janssens K, Verloo P, Ogunjimi B, Hemelsoet D; Program for Undiagnosed Rare Diseases (UD-PrOZA); Hoste L, Roels L, De Bruyne M, De Baere E, Van Dorpe J, Dendooven A, Sieben A, Rice GI, Kerre T, Beyaert R, Uggenti C, Crow YJ, Tavernier SJ, Maelfait J, Haerynck F. Mutations in RNU7-1 Weaken Secondary RNA Structure, Induce MCP-1 and CXCL10 in CSF, and Result in Aicardi-Goutieres Syndrome with Severe End-Organ Involvement. J Clin Immunol. 2022 Jul;42(5):962-974. doi: 10.1007/s10875-022-01209-5. Epub 2022 Mar 23.
- Uggenti C, Lepelley A, Depp M, Badrock AP, Rodero MP, El-Daher MT, Rice GI, Dhir S, Wheeler AP, Dhir A, Albawardi W, Fremond ML, Seabra L, Doig J, Blair N, Martin-Niclos MJ, Della Mina E, Rubio-Roldan A, Garcia-Perez JL, Sproul D, Rehwinkel J, Hertzog J, Boland-Auge A, Olaso R, Deleuze JF, Baruteau J, Brochard K, Buckley J, Cavallera V, Cereda C, De Waele LMH, Dobbie A, Doummar D, Elmslie F, Koch-Hogrebe M, Kumar R, Lamb K, Livingston JH, Majumdar A, Lorenco CM, Orcesi S, Peudenier S, Rostasy K, Salmon CA, Scott C, Tonduti D, Touati G, Valente M, van der Linden H Jr, Van Esch H, Vermelle M, Webb K, Jackson AP, Reijns MAM, Gilbert N, Crow YJ. cGAS-mediated induction of type I interferon due to inborn errors of histone pre-mRNA processing. Nat Genet. 2020 Dec;52(12):1364-1372. doi: 10.1038/s41588-020-00737-3. Epub 2020 Nov 23.
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