Property Summary

NCBI Gene PubMed Count 40
PubMed Score 94.40
PubTator Score 130.16

Knowledge Summary


No data available


  Disease (4)

Disease Target Count
osteosarcoma 7933
Disease Target Count P-value
ulcerative colitis 2087 8.8e-03
medulloblastoma, large-cell 6234 2.6e-02
aldosterone-producing adenoma 664 2.6e-02
Disease Target Count Z-score Confidence
Carcinoma 2147 0.0 1.0


  Differential Expression (4)

Disease log2 FC p
osteosarcoma 1.889 2.0e-05
medulloblastoma, large-cell -1.200 2.6e-02
ulcerative colitis -1.195 8.8e-03
aldosterone-producing adenoma -1.159 2.6e-02

Gene RIF (23)

26872363 P26s4 Associates with the C-Terminal Region of TOPORS.
22972498 Data suggest that TOPORS plays key role in turnover of H2AX (H2A histone family, member X protein) depending on the type of oxidative stress/DNA damage; TOPORS may act as an E3 ubiquitin ligase for histones.
21624951 Disturbed flow induces peroxynitrite production and binding to the E3 SUMO (small ubiquitin-like modifier) ligase PIASy (protein inhibitor of activated STATy).
21159800 In photoreceptors, TOPORS localizes primarily to the basal bodies of connecting cilium and in the centrosomes.
20379614 Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator)
19821153 Polo-like kinase 1 (Plk1)-associated phosphorylation of Topors at S718 is essential for nocodazole-induced degradation of Topors.
19473992 Plk1 modulates Topors activity in suppressing p53 function
19373681 Mutations in TOPORS cause autosomal dominant retinitis pigmentosa (adRP).
19373681 Observational study of gene-disease association. (HuGE Navigator)
19183411 We present a novel mutation in the TOPORS gene co-segregating with a distinct phenotype of adPRD in a large Norwegian family.
18509552 Point mutations and small insertions or deletions in TOPORS cause approximately 1% of autosomal dominant retinitis pigmentosa.
18077445 NKX3.1 can be ubiquitinated by TOPORS in vitro and in vivo, and overexpression of TOPORS leads to NKX3.1 proteasomal degradation in prostate cancer cells
17976381 Topors enhances the formation of high-molecular weight SUMO-1 conjugates of TOP1 in a reconstituted in vitro system and also in human osteosarcoma cells
17924349 Mutations in the gene for topoisomerase I-binding RS protein (TOPORS) in patients with autosomal dominant retinitis pigmentosa (adRP) linked to chromosome 9p21.1 (locus RP31), is reported.
17803295 The findings suggest that TOPORS may function as a tumor suppressor by regulating mSin3A and other proteins involved in chromatin modification.
16122737 Data show that Topors enhances the conjugation of the small ubiquitin-like modifier 1 (SUMO-1) to p53 in vivo and in a reconstituted in vitro system.
15735665 topors mediates p53-dependent cellular responses induced by DNA damage, suggesting its physiological role as a tumor suppressor
15699047 Results show that the ATR/checkpoint kinase 1 pathway plays a predominant role in the response to topoisomerase I inhibitors in carcinoma cells.
15247280 topors has a role as an E3 ubiquitin ligase with specific E2 enzymes and ubiquitinates p53
15107820 topors functions as a negative regulator of cell growth, and possibly as a tumor suppressor
14516784 Multiple SUMO-1 modified forms of Topors could be detected after cotransfection of exogenous SUMO-1 and Topors induced the colocalization of a YFP tagged SUMO-1 protein in a speckled pattern in the nucleus.
12083797 topors localizes in punctate nuclear regions associated with PML protein nuclear bodies.
11842245 Topors, a p53 and topoisomerase I binding protein, interacts with the adeno-associated virus (AAV-2) Rep78/68 proteins and enhances AAV-2 gene expression

AA Sequence


Text Mined References (51)

PMID Year Title
26872363 2016 TOPORS, a Dual E3 Ubiquitin and Sumo1 Ligase, Interacts with 26 S Protease Regulatory Subunit 4, Encoded by the PSMC1 Gene.
25772364 2015 SUMO-2 Orchestrates Chromatin Modifiers in Response to DNA Damage.
25755297 2015 System-wide Analysis of SUMOylation Dynamics in Response to Replication Stress Reveals Novel Small Ubiquitin-like Modified Target Proteins and Acceptor Lysines Relevant for Genome Stability.
25218447 2014 Uncovering global SUMOylation signaling networks in a site-specific manner.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
23186163 2013 Toward a comprehensive characterization of a human cancer cell phosphoproteome.
22972498 2012 TOPORS modulates H2AX discriminating genotoxic stresses.
22610502 2012 Genome-wide analysis of polymorphisms associated with cytokine responses in smallpox vaccine recipients.
21624951 2011 Disturbed flow: p53 SUMOylation in the turnover of endothelial cells.
21406692 2011 System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.
21159800 2011 TOPORS, implicated in retinal degeneration, is a cilia-centrosomal protein.
20379614 Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score.
20188669 2010 SUMOylation-dependent localization of IKKepsilon in PML nuclear bodies is essential for protection against DNA-damage-triggered cell death.
20068231 2010 Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.
19821153 2010 Plk1 phosphorylation of Topors is involved in its degradation.
19690332 2009 Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions.
19473992 2009 Plk1-mediated phosphorylation of Topors regulates p53 stability.
19413330 2009 Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.
19373681 2009 Mutations in TOPORS: a rare cause of autosomal dominant retinitis pigmentosa in continental Europe?
19183411 2010 Autosomal dominant pericentral retinal dystrophy caused by a novel missense mutation in the TOPORS gene.
19053840 2008 Identification of phosphorylation sites of TOPORS and a role for serine 98 in the regulation of ubiquitin but not SUMO E3 ligase activity.
18669648 2008 A quantitative atlas of mitotic phosphorylation.
18509552 2008 Mutations in the TOPORS gene cause 1% of autosomal dominant retinitis pigmentosa.
18077445 2008 Ubiquitination by TOPORS regulates the prostate tumor suppressor NKX3.1.
17976381 2007 The E3 ligase Topors induces the accumulation of polysumoylated forms of DNA topoisomerase I in vitro and in vivo.
17924349 2007 Mutations in TOPORS cause autosomal dominant retinitis pigmentosa with perivascular retinal pigment epithelium atrophy.
17803295 2007 TOPORS functions as a SUMO-1 E3 ligase for chromatin-modifying proteins.
17081983 2006 Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.
16679534 2006 CGI-55 interacts with nuclear proteins and co-localizes to p80-coilin positive-coiled bodies in the nucleus.
16455055 2006 Evidence for the interaction of the regulatory protein Ki-1/57 with p53 and its interacting proteins.
16344560 2006 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.
16189705 2005 A new locus (RP31) for autosomal dominant retinitis pigmentosa maps to chromosome 9p.
16122737 2005 Topors acts as a SUMO-1 E3 ligase for p53 in vitro and in vivo.
15735665 2005 topors, a p53 and topoisomerase I-binding RING finger protein, is a coactivator of p53 in growth suppression induced by DNA damage.
15699047 2005 The role of checkpoint kinase 1 in sensitivity to topoisomerase I poisons.
15489334 2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15364129 2004 Expression of LUN gene that encodes a novel RING finger protein is correlated with development and progression of non-small cell lung cancer.
15302935 2004 Large-scale characterization of HeLa cell nuclear phosphoproteins.
15247280 2004 Topors functions as an E3 ubiquitin ligase with specific E2 enzymes and ubiquitinates p53.
15231748 2004 Functional proteomics mapping of a human signaling pathway.
15164053 2004 DNA sequence and analysis of human chromosome 9.
15107820 2004 The topoisomerase I- and p53-binding protein topors is differentially expressed in normal and malignant human tissues and may function as a tumor suppressor.
14516784 2003 The DNA topoisomerase I binding protein topors as a novel cellular target for SUMO-1 modification: characterization of domains necessary for subcellular localization and sumolation.
12878157 2003 Redifferentiation of dedifferentiated chondrocytes and chondrogenesis of human bone marrow stromal cells via chondrosphere formation with expression profiling by large-scale cDNA analysis.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12083797 2002 The topoisomerase I-binding RING protein, topors, is associated with promyelocytic leukemia nuclear bodies.
11842245 2002 Topors, a p53 and topoisomerase I binding protein, interacts with the adeno-associated virus (AAV-2) Rep78/68 proteins and enhances AAV-2 gene expression.
11278651 2001 Cloning and characterization of LUN, a novel ring finger protein that is highly expressed in lung and specifically binds to a palindromic sequence.
10415337 1999 Identification of a novel gene encoding a p53-associated protein.
10352183 1999 Interaction between human topoisomerase I and a novel RING finger/arginine-serine protein.
8889548 1996 Normalization and subtraction: two approaches to facilitate gene discovery.