Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation

Search Page

Filters

My Custom Filters

Edit custom filters

Results by year

Table representation of search results timeline featuring number of search results per year.

Year Number of Results
1989 1
1996 1
2004 1
2005 1
2006 2
2007 2
2008 3
2010 3
2011 5
2013 3
2014 1
2015 1
2016 1
2017 1
2019 2
2020 2
2021 2
2022 3
2023 8
2024 2
2025 1
2026 0

Publication date

Text availability

Article attribute

Article type

Additional filters

Article Language

Species

Sex

Age

Other

Search Results

43 results

Results by year

Filters applied: . Clear all
Page 1
Structural basis of pre-tRNA intron removal by human tRNA splicing endonuclease.
Zhang X, Yang F, Zhan X, Bian T, Xing Z, Lu Y, Shi Y. Zhang X, et al. Mol Cell. 2023 Apr 20;83(8):1328-1339.e4. doi: 10.1016/j.molcel.2023.03.015. Epub 2023 Apr 6. Mol Cell. 2023. PMID: 37028420 Free article.
In humans, this process is mediated by the tRNA splicing endonuclease (TSEN) comprising four subunits: TSEN2, TSEN15, TSEN34, and TSEN54. ...The mature domain of pre-tRNA is recognized by conserved structural elements of TSEN34, TSEN54, and TSEN …
In humans, this process is mediated by the tRNA splicing endonuclease (TSEN) comprising four subunits: TSEN2, TSEN15, …
Spliceosome-mediated RNA trans-splicing.
Yang Y, Walsh CE. Yang Y, et al. Mol Ther. 2005 Dec;12(6):1006-12. doi: 10.1016/j.ymthe.2005.09.006. Epub 2005 Oct 12. Mol Ther. 2005. PMID: 16226059 Free article. Review.
Unlike conventional gene therapy, in which exogenous cDNAs are introduced into cells, RNA repair approaches, which are based on spliceosome-mediated pre-mRNA trans-splicing, trans-splicing ribozymes, and tRNA-splicing endonuclease, allow the correction of end …
Unlike conventional gene therapy, in which exogenous cDNAs are introduced into cells, RNA repair approaches, which are based on spliceosome- …
Structural basis for pre-tRNA recognition and processing by the human tRNA splicing endonuclease complex.
Hayne CK, Butay KJU, Stewart ZD, Krahn JM, Perera L, Williams JG, Petrovitch RM, Deterding LJ, Matera AG, Borgnia MJ, Stanley RE. Hayne CK, et al. Nat Struct Mol Biol. 2023 Jun;30(6):824-833. doi: 10.1038/s41594-023-00991-z. Epub 2023 May 25. Nat Struct Mol Biol. 2023. PMID: 37231153 Free PMC article.
In eukaryotes, tRNA splicing is initiated by the heterotetrameric tRNA splicing endonuclease (TSEN) complex. All TSEN subunits are essential, and mutations within the complex are associated with a family of neurodevelopmental disorders known as pontocerebella …
In eukaryotes, tRNA splicing is initiated by the heterotetrameric tRNA splicing endonuclease (TSEN) complex. All TSEN s …
Recognition and cleavage mechanism of intron-containing pre-tRNA by human TSEN endonuclease complex.
Yuan L, Han Y, Zhao J, Zhang Y, Sun Y. Yuan L, et al. Nat Commun. 2023 Sep 28;14(1):6071. doi: 10.1038/s41467-023-41845-y. Nat Commun. 2023. PMID: 37770519 Free PMC article.
Here we present a 3.2 A-resolution cryo-EM structure of an active human tRNA splicing endonuclease complex bound to an intron-containing pre-tRNA. TSEN54, along with the unique regions of TSEN34 and TSEN2, cooperatively recognizes the mature body of pr …
Here we present a 3.2 A-resolution cryo-EM structure of an active human tRNA splicing endonuclease complex bound to an …
New insights into RNA processing by the eukaryotic tRNA splicing endonuclease.
Hayne CK, Sekulovski S, Hurtig JE, Stanley RE, Trowitzsch S, van Hoof A. Hayne CK, et al. J Biol Chem. 2023 Sep;299(9):105138. doi: 10.1016/j.jbc.2023.105138. Epub 2023 Aug 5. J Biol Chem. 2023. PMID: 37544645 Free PMC article. Review.
Through its role in intron cleavage, tRNA splicing endonuclease (TSEN) plays a critical function in the maturation of intron-containing pre-tRNAs. ...
Through its role in intron cleavage, tRNA splicing endonuclease (TSEN) plays a critical function in the maturation of i …
What connects splicing of transfer RNA precursor molecules with pontocerebellar hypoplasia?
Sekulovski S, Trowitzsch S. Sekulovski S, et al. Bioessays. 2023 Feb;45(2):e2200130. doi: 10.1002/bies.202200130. Epub 2022 Dec 14. Bioessays. 2023. PMID: 36517085 Review.
Aberrations in the extensive tRNA biogenesis pathways lead to severe neurological disorders in humans. Mutations in the tRNA splicing endonuclease (TSEN) and its associated RNA kinase cleavage factor polyribonucleotide kinase subunit 1 (CLP1) cause pontocereb …
Aberrations in the extensive tRNA biogenesis pathways lead to severe neurological disorders in humans. Mutations in the tRNA splic …
Reconstitution of the human tRNA splicing endonuclease complex: insight into the regulation of pre-tRNA cleavage.
Hayne CK, Schmidt CA, Haque MI, Matera AG, Stanley RE. Hayne CK, et al. Nucleic Acids Res. 2020 Aug 20;48(14):7609-7622. doi: 10.1093/nar/gkaa438. Nucleic Acids Res. 2020. PMID: 32476018 Free PMC article.
In archaea and eukaryotes, tRNA intron removal is catalyzed by the tRNA splicing endonuclease (TSEN) complex. Eukaryotic TSEN is comprised of four core subunits (TSEN54, TSEN2, TSEN34 and TSEN15). ...
In archaea and eukaryotes, tRNA intron removal is catalyzed by the tRNA splicing endonuclease (TSEN) complex. Eukaryoti …
Structural basis of substrate recognition by human tRNA splicing endonuclease TSEN.
Sekulovski S, Sušac L, Stelzl LS, Tampé R, Trowitzsch S. Sekulovski S, et al. Nat Struct Mol Biol. 2023 Jun;30(6):834-840. doi: 10.1038/s41594-023-00992-y. Epub 2023 May 25. Nat Struct Mol Biol. 2023. PMID: 37231152
Heterotetrameric human transfer RNA (tRNA) splicing endonuclease TSEN catalyzes intron excision from precursor tRNAs (pre-tRNAs), utilizing two composite active sites. ...
Heterotetrameric human transfer RNA (tRNA) splicing endonuclease TSEN catalyzes intron excision from precursor tRNAs (p …
Clinical, neuroradiological and genetic findings in pontocerebellar hypoplasia.
Namavar Y, Barth PG, Kasher PR, van Ruissen F, Brockmann K, Bernert G, Writzl K, Ventura K, Cheng EY, Ferriero DM, Basel-Vanagaite L, Eggens VR, Krägeloh-Mann I, De Meirleir L, King M, Graham JM Jr, von Moers A, Knoers N, Sztriha L, Korinthenberg R; PCH Consortium; Dobyns WB, Baas F, Poll-The BT. Namavar Y, et al. Brain. 2011 Jan;134(Pt 1):143-56. doi: 10.1093/brain/awq287. Epub 2010 Oct 15. Brain. 2011. PMID: 20952379 Free PMC article.
Mutations in the transfer RNA splicing endonuclease subunit genes (TSEN54, TSEN2, TSEN34) were found to be associated with pontocerebellar hypoplasia types 2 and 4. Mutations in the mitochondrial transfer RNA arginyl synthetase gene (RARS2) were associated with pontocerebe …
Mutations in the transfer RNA splicing endonuclease subunit genes (TSEN54, TSEN2, TSEN34) were found to be associated with pontocereb …
Molecular and neuroimaging findings in pontocerebellar hypoplasia type 2 (PCH2): is prenatal diagnosis possible?
Graham JM Jr, Spencer AH, Grinberg I, Niesen CE, Platt LD, Maya M, Namavar Y, Baas F, Dobyns WB. Graham JM Jr, et al. Am J Med Genet A. 2010 Sep;152A(9):2268-76. doi: 10.1002/ajmg.a.33579. Am J Med Genet A. 2010. PMID: 20803644 Free PMC article.
Most patients with PCH2 have mutations in TSEN54, with occasional mutations found in TSEN34 or TSEN2, genes that encode subunits of tRNA splicing endonuclease. Although this is a congenital disorder of pontocerebellar dysgenesis with fetal onset of neu …
Most patients with PCH2 have mutations in TSEN54, with occasional mutations found in TSEN34 or TSEN2, genes that encode subunits of …
43 results