TSEN34 Gene

HGNC Family	tRNA-splicing endonuclease subunits (TSEN)
Name	TSEN34 tRNA splicing endonuclease subunit
Description	This gene encodes a catalytic subunit of the tRNA splicing endonuclease, which catalyzes the removal of introns from precursor tRNAs. The endonuclease complex is also associated with a pre-mRNA 3-prime end processing factor. A mutation in this gene results in the neurological disorder pontocerebellar hypoplasia type 2. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Oct 2009]
Summary	{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nAlthough none of the provided abstracts directly mention TSEN34, they collectively describe a network of developmental and cellular regulatory processes that offer useful context for understanding how TSEN34’s canonical function—its role as a tRNA splicing endonuclease subunit—is positioned within the broader framework of gene expression and cell homeostasis. Several studies focus on mechanisms controlling transcription factor expression and chromatin remodeling during embryogenesis, such as the proper regulation of Hox genes by retinoic acid, enhancer activity, and autoregulatory circuits that establish segment polarity (for example, see."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "1", "end_ref": "7"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nOther studies provided here detail the contribution of cytoskeletal regulators and enzymatic activities to cellular differentiation, morphogenesis, and organ function. For instance, work on actin-based motor proteins such as MYO9A has highlighted its role in neuromuscular junction function and epithelial organization, while analyses of transcription factors like Hoxd4 reveal their impact on chondrocyte differentiation and skeletal patterning."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "8", "end_ref": "12"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nAdditional research in these abstracts emphasizes the importance of lipid-modifying enzymes in homeostatic processes and disease; for example, intestinal alkaline sphingomyelinase (alk‐SMase) has been shown to affect cholesterol absorption and modulate inflammatory responses in colitis, thereby influencing tumorigenesis and tissue remodeling."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "13", "end_ref": "16"}]}, {"type": "t", "text": " While TSEN34—the endonuclease subunit involved in precursor tRNA splicing and thereby essential for accurate mRNA translation—is not discussed in any of these studies, its fundamental role in RNA maturation implies that TSEN34-dependent tRNA processing is a critical underpinning for all protein synthesis. In this way, TSEN34-mediated pathways may intersect with diverse cellular processes such as those governing transcriptional regulation, cytoskeletal dynamics, and metabolic responses highlighted throughout the literature.\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Emma C Collins, Alexandre Appert, Linda Ariza-McNaughton, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Mouse Af9 is a controller of embryo patterning, like Mll, whose human homologue fuses with Af9 after chromosomal translocation in leukemia."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cell Biol (2002)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1128/MCB.22.20.7313-7324.2002"}], "href": "https://doi.org/10.1128/MCB.22.20.7313-7324.2002"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "12242306"}], "href": "https://pubmed.ncbi.nlm.nih.gov/12242306"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Christof Nolte, Angel Amores, Erzsébet Nagy Kovács, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The role of a retinoic acid response element in establishing the anterior neural expression border of Hoxd4 transgenes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mech Dev (2003)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/s0925-4773(02)00442-2"}], "href": "https://doi.org/10.1016/s0925-4773(02"}, {"type": "t", "text": "00442-2) PMID: "}, {"type": "a", "children": [{"type": "t", "text": "12591602"}], "href": "https://pubmed.ncbi.nlm.nih.gov/12591602"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Mojgan Rastegar, Laila Kobrossy, Erzsebet Nagy Kovacs, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Sequential histone modifications at Hoxd4 regulatory regions distinguish anterior from posterior embryonic compartments."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cell Biol (2004)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1128/MCB.24.18.8090-8103.2004"}], "href": "https://doi.org/10.1128/MCB.24.18.8090-8103.2004"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15340071"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15340071"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Patricia Serpente, Stefan Tümpel, Norbert B Ghyselinck, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Direct crossregulation between retinoic acid receptor {beta} and Hox genes during hindbrain segmentation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Development (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/dev.01593"}], "href": "https://doi.org/10.1242/dev.01593"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15634700"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15634700"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Laila Kobrossy, Mojgan Rastegar, Mark Featherstone "}, {"type": "b", "children": [{"type": "t", "text": "Interplay between chromatin and trans-acting factors regulating the Hoxd4 promoter during neural differentiation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Chem (2006)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1074/jbc.M602555200"}], "href": "https://doi.org/10.1074/jbc.M602555200"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16757478"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16757478"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Christof Nolte, Mojgan Rastegar, Angel Amores, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Stereospecificity and PAX6 function direct Hoxd4 neural enhancer activity along the antero-posterior axis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Dev Biol (2006)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.ydbio.2006.08.061"}], "href": "https://doi.org/10.1016/j.ydbio.2006.08.061"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "17010333"}], "href": "https://pubmed.ncbi.nlm.nih.gov/17010333"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Sze Lynn Calista Phua, V Sivakamasundari, Yu Shao, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Nuclear accumulation of an uncapped RNA produced by Drosha cleavage of a transcript encoding miR-10b and HOXD4."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2011)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0025689"}], "href": "https://doi.org/10.1371/journal.pone.0025689"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21991333"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21991333"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Marouan Abouhamed, Kay Grobe, Isabelle V Leefa Chong San, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Myosin IXa regulates epithelial differentiation and its deficiency results in hydrocephalus."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Biol Cell (2009)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1091/mbc.e09-04-0291"}], "href": "https://doi.org/10.1091/mbc.e09-04-0291"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "19828736"}], "href": "https://pubmed.ncbi.nlm.nih.gov/19828736"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Claudia Kruger, Claudia Kappen "}, {"type": "b", "children": [{"type": "t", "text": "Expression of cartilage developmental genes in Hoxc8- and Hoxd4-transgenic mice."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2010)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0008978"}], "href": "https://doi.org/10.1371/journal.pone.0008978"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "20126390"}], "href": "https://pubmed.ncbi.nlm.nih.gov/20126390"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Ying Chen, Ping Zhang, Shu-Chang Xu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Enhanced colonic tumorigenesis in alkaline sphingomyelinase (NPP7) knockout mice."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Mol Cancer Ther (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1158/1535-7163.MCT-14-0468-T"}], "href": "https://doi.org/10.1158/1535-7163.MCT-14-0468-T"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25381265"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25381265"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Sabine Thelen, Marouan Abouhamed, Giuliano Ciarimboli, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Rho GAP myosin IXa is a regulator of kidney tubule function."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Physiol Renal Physiol (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1152/ajprenal.00220.2014"}], "href": "https://doi.org/10.1152/ajprenal.00220.2014"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26136556"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26136556"}]}, {"type": "r", "ref": 12, "children": [{"type": "t", "text": "Emily O'Connor, Vietxuan Phan, Isabell Cordts, et al. "}, {"type": "b", "children": [{"type": "t", "text": "MYO9A deficiency in motor neurons is associated with reduced neuromuscular agrin secretion."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddy054"}], "href": "https://doi.org/10.1093/hmg/ddy054"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29462312"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29462312"}]}, {"type": "r", "ref": 13, "children": [{"type": "t", "text": "Ping Zhang, Ying Chen, Yajun Cheng, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Alkaline sphingomyelinase (NPP7) promotes cholesterol absorption by affecting sphingomyelin levels in the gut: A study with NPP7 knockout mice."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Physiol Gastrointest Liver Physiol (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1152/ajpgi.00319.2013"}], "href": "https://doi.org/10.1152/ajpgi.00319.2013"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24650549"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24650549"}]}, {"type": "r", "ref": 14, "children": [{"type": "t", "text": "Ping Zhang, Ying Chen, Tao Zhang, et al. 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"}, {"type": "b", "children": [{"type": "t", "text": "Alkaline sphingomyelinase (NPP7) impacts the homeostasis of intestinal T lymphocyte populations."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Front Immunol (2023)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3389/fimmu.2022.1050625"}], "href": "https://doi.org/10.3389/fimmu.2022.1050625"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "36741374"}], "href": "https://pubmed.ncbi.nlm.nih.gov/36741374"}]}, {"type": "r", "ref": 16, "children": [{"type": "t", "text": "Ye Tian, Xin Li, Xu Wang, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Alkaline sphingomyelinase deficiency impairs intestinal mucosal barrier integrity and reduces antioxidant capacity in dextran sulfate sodium-induced colitis."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "World J Gastroenterol (2024)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3748/wjg.v30.i10.1405"}], "href": "https://doi.org/10.3748/wjg.v30.i10.1405"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "38596488"}], "href": "https://pubmed.ncbi.nlm.nih.gov/38596488"}]}]}]}
Synonyms	PCH2C, SEN34L, LENG5, SEN34
Proteins	SEN34_HUMAN
NCBI Gene ID	79042
API
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Predicted Functions
Co-expressed Genes
Expression in Tissues and Cell Lines

Functional Associations

TSEN34 has 5,863 functional associations with biological entities spanning 9 categories (molecular profile, organism, chemical, disease, phenotype or trait, functional term, phrase or reference, structural feature, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 115 datasets.

Click the + buttons to view associations for TSEN34 from the datasets below.

If available, associations are ranked by standardized value

Harmonizome 3.0

TSEN34 Gene

Functional Associations

Please acknowledge the Harmonizome in your publications by citing the following reference(s):

	Dataset	Summary
	Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of TSEN34 gene relative to other tissues from the Allen Brain Atlas Adult Human Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles	tissues with high or low expression of TSEN34 gene relative to other tissues from the Allen Brain Atlas Adult Mouse Brain Tissue Gene Expression Profiles dataset.
	Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles	tissue samples with high or low expression of TSEN34 gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray	tissue samples with high or low expression of TSEN34 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by Microarray dataset.
	Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq	tissue samples with high or low expression of TSEN34 gene relative to other tissue samples from the Allen Brain Atlas Developing Human Brain Tissue Gene Expression Profiles by RNA-seq dataset.
	Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles	tissues with high or low expression of TSEN34 gene relative to other tissues from the Allen Brain Atlas Prenatal Human Brain Tissue Gene Expression Profiles dataset.
	BioGPS Cell Line Gene Expression Profiles	cell lines with high or low expression of TSEN34 gene relative to other cell lines from the BioGPS Cell Line Gene Expression Profiles dataset.
	BioGPS Human Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of TSEN34 gene relative to other cell types and tissues from the BioGPS Human Cell Type and Tissue Gene Expression Profiles dataset.
	BioGPS Mouse Cell Type and Tissue Gene Expression Profiles	cell types and tissues with high or low expression of TSEN34 gene relative to other cell types and tissues from the BioGPS Mouse Cell Type and Tissue Gene Expression Profiles dataset.
	Carcinogenome Chemical Perturbation Carcinogenicity Signatures	small molecule perturbations changing expression of TSEN34 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
	CCLE Cell Line Gene CNV Profiles	cell lines with high or low copy number of TSEN34 gene relative to other cell lines from the CCLE Cell Line Gene CNV Profiles dataset.
	CCLE Cell Line Gene Expression Profiles	cell lines with high or low expression of TSEN34 gene relative to other cell lines from the CCLE Cell Line Gene Expression Profiles dataset.
	CCLE Cell Line Proteomics	Cell lines associated with TSEN34 protein from the CCLE Cell Line Proteomics dataset.
	ChEA Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of TSEN34 gene from the CHEA Transcription Factor Binding Site Profiles dataset.
	ChEA Transcription Factor Targets	transcription factors binding the promoter of TSEN34 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets dataset.
	ChEA Transcription Factor Targets 2022	transcription factors binding the promoter of TSEN34 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
	ClinVar Gene-Phenotype Associations 2025	phenotypes associated with TSEN34 gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
	CM4AI KOLF21J CRISPRi Gene Perturbation Atlas	gene perturbations changing expression of TSEN34 gene from the CM4AI KOLF21J CRISPRi Gene Perturbation Atlas dataset.
	CM4AI U2OS Cell Map Protein Localization Assemblies	assemblies containing TSEN34 protein from integrated AP-MS and IF data from the CM4AI U2OS Cell Map Protein Localization Assemblies dataset.
	CMAP Signatures of Differentially Expressed Genes for Small Molecules	small molecule perturbations changing expression of TSEN34 gene from the CMAP Signatures of Differentially Expressed Genes for Small Molecules dataset.
	COMPARTMENTS Curated Protein Localization Evidence Scores	cellular components containing TSEN34 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores	cellular components co-occuring with TSEN34 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores dataset.
	COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025	cellular components co-occuring with TSEN34 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
	CORUM Protein Complexes	protein complexs containing TSEN34 protein from the CORUM Protein Complexes dataset.
	COSMIC Cell Line Gene CNV Profiles	cell lines with high or low copy number of TSEN34 gene relative to other cell lines from the COSMIC Cell Line Gene CNV Profiles dataset.
	COSMIC Cell Line Gene Mutation Profiles	cell lines with TSEN34 gene mutations from the COSMIC Cell Line Gene Mutation Profiles dataset.
	CTD Gene-Disease Associations	diseases associated with TSEN34 gene/protein from the curated CTD Gene-Disease Associations dataset.
	DeepCoverMOA Drug Mechanisms of Action	small molecule perturbations with high or low expression of TSEN34 protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
	DISEASES Curated Gene-Disease Association Evidence Scores 2025	diseases involving TSEN34 gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
	DISEASES Experimental Gene-Disease Association Evidence Scores 2025	diseases associated with TSEN34 gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores	diseases co-occuring with TSEN34 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores dataset.
	DISEASES Text-mining Gene-Disease Association Evidence Scores 2025	diseases co-occuring with TSEN34 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
	DisGeNET Gene-Disease Associations	diseases associated with TSEN34 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
	DisGeNET Gene-Phenotype Associations	phenotypes associated with TSEN34 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
	ENCODE Histone Modification Site Profiles	histone modification site profiles with high histone modification abundance at TSEN34 gene from the ENCODE Histone Modification Site Profiles dataset.
	ENCODE Transcription Factor Binding Site Profiles	transcription factor binding site profiles with transcription factor binding evidence at the promoter of TSEN34 gene from the ENCODE Transcription Factor Binding Site Profiles dataset.
	ENCODE Transcription Factor Targets	transcription factors binding the promoter of TSEN34 gene in ChIP-seq datasets from the ENCODE Transcription Factor Targets dataset.
	ESCAPE Omics Signatures of Genes and Proteins for Stem Cells	PubMedIDs of publications reporting gene signatures containing TSEN34 from the ESCAPE Omics Signatures of Genes and Proteins for Stem Cells dataset.
	GDSC Cell Line Gene Expression Profiles	cell lines with high or low expression of TSEN34 gene relative to other cell lines from the GDSC Cell Line Gene Expression Profiles dataset.
	GeneSigDB Published Gene Signatures	PubMedIDs of publications reporting gene signatures containing TSEN34 from the GeneSigDB Published Gene Signatures dataset.