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TRNA splicing endonuclease 54 homolog

Sen54, Sen54p, HsSen54, PCH4
This gene encodes a subunit of the tRNA splicing endonuclease complex, which catalyzes the removal of introns from precursor tRNAs. The complex is also implicated in pre-mRNA 3-prime end processing. Mutations in this gene result in pontocerebellar hypoplasia type 2.[provided by RefSeq, Oct 2009] (from NCBI)
Top mentioned proteins: sen2, HsSen15, Sen34, PCH6, CAN
Papers on Sen54
Assessing the performance of the photo-acoustic infrared gas monitor for measuring CO(2), N(2)O, and CH(4) fluxes in two major cereal rotations.
Ladha et al., New Delhi, India. In Glob Chang Biol, 2014
All the PAS readings were corrected for baseline drifts over time and PAS-CH4 (PCH4 ) readings in flooded rice were corrected for water vapor interferences.
Pontocerebellar hypoplasia type 2 and TSEN2: review of the literature and two novel mutations.
Kutsche et al., Hamburg, Germany. In Eur J Med Genet, 2013
Seven different subtypes have been reported (PCH1-7) and mutations in three genes, TSEN2, TSEN34 and TSEN54 encoding three of four subunits of the tRNA splicing endonuclease complex have been found to underlie PCH2, PCH4 and PCH5.
Expression of Nur77 induced by an n-butylidenephthalide derivative promotes apoptosis and inhibits cell growth in oral squamous cell carcinoma.
Harn et al., Taiwan. In Invest New Drugs, 2012
PCH4, a derivative of n-butylidenephthalide, has been investigated for its anti-tumor effects on oral squamous cell carcinoma (OSCC).
TSEN54 mutations cause pontocerebellar hypoplasia type 5.
Baas et al., Amsterdam, Netherlands. In Eur J Hum Genet, 2011
Mutations in the tRNA splicing endonuclease subunit (TSEN) genes 54, 2 and 34 are found in PCH2 and PCH4.
TSEN54 mutation in a child with pontocerebellar hypoplasia type 1.
Santorelli et al., In Acta Neuropathol, 2011
TSEN54 mutation causes a severe form of pontocerebellar hypoplasia type 1 in a family.
Impairment of the tRNA-splicing endonuclease subunit 54 (tsen54) gene causes neurological abnormalities and larval death in zebrafish models of pontocerebellar hypoplasia.
Baas et al., Amsterdam, Netherlands. In Hum Mol Genet, 2011
The majority of PCH2 and PCH4 cases are caused by mutations in the TSEN54 gene; one of the four subunits comprising the tRNA-splicing endonuclease (TSEN) complex.
Overexpression of the orphan receptor Nur77 and its translocation induced by PCH4 may inhibit malignant glioma cell growth and induce cell apoptosis.
Chiou et al., Taiwan. In J Surg Oncol, 2011
The anti-GBM cell effects of one derivative of BP, (Z)-N-(2-(dimethylamino)ethyl)-2-(3-((3-oxoisobenzofuran-1(3H)-ylidene)methyl)phenoxy)acetamide (PCH4) were tested in vitro and in vivo.
Pontocerebellar hypoplasia: review of classification and genetics, and exclusion of several genes known to be important for cerebellar development.
Zoghbi et al., Cleveland, United States. In J Child Neurol, 2011
The results demonistrated that not all cases of clinically defined pontocerebellar hypoplasia-4 result from mutations in TSEN54.
Classification, diagnosis and potential mechanisms in pontocerebellar hypoplasia.
Baas et al., Amsterdam, Netherlands. In Orphanet J Rare Dis, 2010
Mutations in three tRNA splicing endonuclease subunit genes were found to be responsible for PCH2, PCH4 and PCH5.
Pontocerebellar hypoplasia: clinical, pathologic, and genetic studies.
Bertini et al., Genova, Italy. In Neurology, 2010
We confirm that the common p.A307S mutation in TSEN54 is responsible for most of the patients with a PCH2 phenotype.
Pontocerebellar hypoplasia type 6: A British case with PEHO-like features.
Brown et al., Exeter, United Kingdom. In Am J Med Genet A, 2010
Six subtypes of autosomal recessive pontocerebellar hypoplasia (PCH) have been identified and the genetic basis of four of these (PCH1, PCH2, PCH4, and PCH6) is known.
TSEN54-Related Pontocerebellar Hypoplasia
Baas et al., Seattle, United States. In Unknown Journal, 2009
CLINICAL CHARACTERISTICS: TSEN54-related pontocerebellar hypoplasia (PCH) includes three PCH types (PCH2, 4, and 5) that share characteristic neuroradiologic and neurologic findings.
tRNA splicing endonuclease mutations cause pontocerebellar hypoplasia.
Baas et al., Köln, Germany. In Nat Genet, 2008
In two subtypes, PCH2 and PCH4, we identified mutations in three of the four different subunits of the tRNA-splicing endonuclease complex.
Three-dimensional structure determined for a subunit of human tRNA splicing endonuclease (Sen15) reveals a novel dimeric fold.
Markley et al., Madison, United States. In J Mol Biol, 2007
Splicing of eukaryal intron-containing tRNAs requires the action of the heterotetrameric splicing endonuclease, which is composed of two catalytic subunits, Sen34 and Sen2, and two structural subunits, Sen15 and Sen54.
Cleavage of pre-tRNAs by the splicing endonuclease requires a composite active site.
Peltz et al., South Plainfield, United States. In Nature, 2006
The first step of splicing, intron recognition and cleavage, is performed by the tRNA-splicing endonuclease, a tetrameric enzyme composed of the protein subunits Sen54, Sen2, Sen34 and Sen15.
Identification of a human endonuclease complex reveals a link between tRNA splicing and pre-mRNA 3' end formation.
Trotta et al., South Plainfield, United States. In Cell, 2004
This enzyme consists of HsSen2, HsSen34, HsSen15, and HsSen54, homologs of the yeast tRNA endonuclease subunits.
Possibility of cytoplasmic pre-tRNA splicing: the yeast tRNA splicing endonuclease mainly localizes on the mitochondria.
Endo et al., Nagoya, Japan. In Mol Biol Cell, 2003
In yeast, the tRNA splicing endonuclease that cleaves the exon-intron junctions of pre-tRNAs consists of Sen54p, Sen2p, Sen34p, and Sen15p and was thought to be an integral membrane protein of the inner nuclear envelope.
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