TOPORS - TOP1 binding arginine/serine rich protein, E3 ubiquitin ligase Gene

Also Known as LUN; RP31; P53BP3; TP53BPL

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 10210

About TOPORS

Cytogenetic location: 9p21.1 Genomic coordinates (GRCh38): 9:32,540,544-32,552,586 (from NCBI)

This gene has 2 transcripts (splice variants), 252 orthologues and is associated with 4 phenotypes. Ubiquitous expression in testis (RPKM 10.5), bone marrow (RPKM 10.0) and 25 other tissues.

Summary

This gene encodes a nuclear protein which is serine and arginine rich, and contains a RING-type zinc finger domain. It is highly expressed in the testis, and functions as an ubiquitin-protein E3 Ligase. Mutations in this gene are associated with retinitis pigmentosa type 31. Alternatively spliced transcript variants, encoding different isoforms, have been observed for this locus. [provided by RefSeq, Sep 2010]

TOPORS Products (2)

mRNA	Protein	Name
NM_001195622.2	NP_001182551.1	E3 ubiquitin-protein ligase Topors isoform 2
NM_005802.5	NP_005793.2	E3 ubiquitin-protein ligase Topors isoform 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables DNA binding	IDA IDA: Inferred from direct assay	11278651	GOA
enables DNA topoisomerase binding	IPI IPI: Inferred from physical interaction	10352183	GOA
enables SUMO transferase activity	EXP EXP: Inferred from Experiment	17803295	GOA
enables SUMO transferase activity	IDA IDA: Inferred from direct assay	17803295	GOA
enables SUMO transferase activity	IMP IMP: Inferred from mutant phenotype	19473992	GOA
enables antigen binding	IPI IPI: Inferred from physical interaction	10415337	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11842245	GOA
enables ubiquitin protein ligase activity	IDA IDA: Inferred from direct assay	15247280	GOA
enables ubiquitin-protein transferase activity	IDA IDA: Inferred from direct assay	18077445	GOA
enables ubiquitin-protein transferase activity	IMP IMP: Inferred from mutant phenotype	19473992	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in DNA damage response	IDA IDA: Inferred from direct assay	15735665	GOA
involved in intrinsic apoptotic signaling pathway in response to DNA damage	IDA IDA: Inferred from direct assay	20188669	GOA
involved in maintenance of protein location in nucleus	IDA IDA: Inferred from direct assay	14516784	GOA
involved in proteasome-mediated ubiquitin-dependent protein catabolic process	IMP IMP: Inferred from mutant phenotype	18077445	GOA
involved in protein K48-linked ubiquitination	IDA IDA: Inferred from direct assay	15247280	GOA
involved in protein localization to nucleus	IMP IMP: Inferred from mutant phenotype	20188669	GOA
involved in protein monoubiquitination	IDA IDA: Inferred from direct assay	15247280	GOA
involved in protein polyubiquitination	IDA IDA: Inferred from direct assay	15247280	GOA
involved in protein sumoylation	IDA IDA: Inferred from direct assay	17803295	GOA
involved in protein sumoylation	IMP IMP: Inferred from mutant phenotype	20188669	GOA
involved in ubiquitin-dependent protein catabolic process	IMP IMP: Inferred from mutant phenotype	19473992	GOA

Cellular Component GO Annotation	Evidence	References	Source
colocalizes with PML body	IDA IDA: Inferred from direct assay	21159800	GOA
located in PML body	IDA IDA: Inferred from direct assay	20188669	GOA
located in centriole	IDA IDA: Inferred from direct assay	21159800	GOA
located in ciliary basal body	IDA IDA: Inferred from direct assay	21159800	GOA
part of gamma-tubulin complex	IDA IDA: Inferred from direct assay	21159800	GOA
located in nuclear speck	IDA IDA: Inferred from direct assay	14516784	GOA
located in nucleus	IDA IDA: Inferred from direct assay	18077445	GOA
located in photoreceptor connecting cilium	IDA IDA: Inferred from direct assay	21159800	GOA
located in spindle pole	IDA IDA: Inferred from direct assay	21159800	GOA
part of ubiquitin ligase complex	IDA IDA: Inferred from direct assay	15247280	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

TOPORS Protein Structure

zf-C3HC4

0
200
400
600
800
1000
1045 a.a.

Protein Preferred Names	Protein Names
E3 ubiquitin-protein ligase Topors	RING-type E3 ubiquitin transferase Topors

TOPORS Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Cross	TOPORS	Q9NS56	Rep68	Adeno-associated virus 2	P03132	Y2H	11842245

Cross: Cross-species interaction Intra: Intraspecies interaction

TOPORS Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P89585	TOPORS Antibody (YA8929)	WB, ICC/IF, IF-Tissue, IHC-P, IP, ELISA	human

Related Diseases

Diseases	Alias
Retinitis Pigmentosa 31	RP31 Retinitis Pigmentosa-31 Retinitis Pigmentosa, Type 31
Retinitis Pigmentosa	RP Rod-Cone Dystrophy Autosomal Recessive Retinitis Pigmentosa Non-Syndromic Retinitis Pigmentosa Pericentral Pigmentary Retinopathy Pigmentary Retinopathy Tapetoretinal Degeneration Rcd Retinitis Pigmentosa Autosomal Recessive ARRP Retinitis Pigmentosa, Autosomal Recessive Retinitis Pigmentosa 1
Orofaciodigital Syndrome Vi	OFD6 Varadi-Papp Syndrome Varadi Syndrome Joubert Syndrome With Orofaciodigital Defect Orofaciodigital Syndrome Type 6 Orofaciodigital Syndrome 6 Oral-Facial-Digital Syndrome, Type Vi Ofds Vi Polydactyly, Cleft Lip/Palate Or Lingual Lump, And Psychomotor Retardation Polydactyly Cleft Lip Palate Psychomotor Retardation Oral-Facial-Digital Syndrome Type 6 Polydactyly-Cleft Lip/Palate-Psychomotor Retardation Syndrome Polydactyly - Cleft Lip/Palate - Psychomotor Retardation Váradi Syndrome Váradi-Papp Syndrome Joubert Syndrome With Oral-Facial-Digital Syndrome Oral-Facial-Digital Syndrome 6 Joubert-Orofaciodigital Syndrome Orofaciodigital Syndrome, Type Vi
Fundus Dystrophy	Retinal Dystrophy Retinal Dystrophies Dystrophy, Retinal
Cone-Rod Dystrophy 2	Cone-Rod Dystrophy CORD2 Cone-Rod Retinal Dystrophy Rcrd2 Cone-Rod Retinal Dystrophy 2 Crd2 Cord Crd Retinal Cone-Rod Dystrophy Cone-Rod Retinal Dystrophy-2 Retinal Cone-Rod Dystrophy 2 Tapetoretinal Degeneration Cone-Rod Degeneration Cone Rod Dystrophy Dystrophy, Cone-Rod Dystrophy, Cone-Rod, Type 2 Retinitis Pigmentosa Retinitis Pigmentosa 2 Progressive Cone-Rod Dystrophy
Retinitis
Cold-Induced Sweating Syndrome 3
Retinitis Pigmentosa 74	RP74 Retinitis Pigmentosa, Type 74
Retinal Degeneration	Degeneration Of Retina
Renal-Hepatic-Pancreatic Dysplasia	Ivemark'S Syndrome Ivemark Ii Syndrome Renohepaticopancreatic Dysplasia
Choroidal Dystrophy, Central Areolar, 1	Choroidal Sclerosis Choroidal Dystrophy Choroidal Dystrophy, Central Areolar Cacd Central Areolar Choroidal Dystrophy CACD1 Choroidal Dystrophy, Central Areolar 1 Choroidal Dystrophy Central Areolar Central Areolar Choroidal Sclerosis Choroidal Degenerations Areolar Atrophy Of The Macula Partial Central Choroid Dystrophy Degenerative Choroidopathy Chorioretinal Degeneration Hereditary Chorioretinal Degeneration Hereditary Degeneration Of Choroid Hereditary Choroidal Dystrophies Generalised Choroidal Dystrophy Hereditary Choroidopathy
Usher Syndrome Type 2	Ush2 Usher Syndrome Type Ii
Usher Syndrome	Deafness-Retinitis Pigmentosa Syndrome Dystrophia Retinae Pigmentosa-Dysostosis Syndrome Graefe-Usher Syndrome Hallgren Syndrome Usher'S Syndrome Retinitis Pigmentosa-Deafness Syndrome Retinitis Pigmentosa-Hearing Loss Syndrome Ush Usher Syndromes
Leber Plus Disease	Leber Congenital Amaurosis Lca Leber'S Amaurosis Leber'S Disease Amaurosis Congenita Of Leber Amaurosis Congenita Of Leber, Type 1 Lhon Plus Disease Congenital Absence Of The Rods And Cones Congenital Retinal Blindness Crb Congenital Amaurosis Of Retinal Origin Leber'S Congenital Amaurosis Leber Congenital Amaurosis 1 Leber'S Congenital Tapetoretinal Degeneration Leber'S Congenital Tapetoretinal Dysplasia Lca1 Leber Congenital Amaurosis Type 1 Retinal Blindness, Congenital Amaurosis, Leber Congenital Dysgenesis Neuroepithelialis Retinae Hereditary Epithelial Dysplasia Of Retina Hereditary Retinal Aplasia Heredoretinopathia Congenitalis Leber Abiotrophy Leber Congenital Tapetoretinal Degeneration Lebers Congenital Amaurosis Optic Atrophy, Hereditary, Leber
Bardet-Biedl Syndrome	Bbs Biedl-Bardet Syndrome

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Clinical Phase
Pre-clinical Phase

Clinical Phase

Bioactive Molecules (6)

Cat. No.	Product Name	Effect	Purity	Phase
HY-164702	BL-B01D1	Inhibitor	99.65%	Phase 3
HY-U00248A	LMP744 hydrochloride	Inhibitor	99.08%	Phase 2
HY-168739	Topoisomerase I inhibitor 17		/	Phase 2
HY-160930	TOP1288	Inhibitor	/	Phase 2
HY-171124	Tilatamig samrotecan	Inhibitor	95.26%	Phase 1
HY-18350	Indimitecan	Inhibitor	98.30%	Phase 1

Inhibitory Antibodies (1)

Cat. No.	Product Name	Effect	Purity	Phase
HY-P990940	Precemtabart	Inhibitor	99.00%	Phase 3

Pre-clinical Phase

Bioactive Molecules (88)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-164734	Raludotatug Deruxtecan		99.29%	Unkown
HY-148819A	NH2-bicyclo[1.1.1]pentane-7-MAD-MDCPT hydrochloride	Inhibitor	98.46%	Unkown
HY-148819	NH2-bicyclo[1.1.1]pentane-7-MAD-MDCPT	Inhibitor	98.71%	Unkown
HY-111630	PluriSIn #2	Inhibitor	98.0%	Unkown
HY-174374	Topobexin	Inhibitor	99.04%	Unkown
HY-118581	Coralyne chloride	Inhibitor	98.39%	Unkown
HY-163099	P5(PEG24)-VC-PAB-Exatecan		99.74%	Unkown
HY-153278	Q901	Inhibitor	98.38%	Unkown
HY-164143	T638	Inhibitor	98.61%	Unkown
HY-U00248	LMP744	Inhibitor	98.33%	Unkown
HY-116785	BRD32048	Inhibitor	99.94%	Unkown
HY-153278A	Q901 TFA	Inhibitor	98.55%	Unkown
HY-160005	LMP517	Inhibitor	99.20%	Unkown
HY-175203	Topi MF-6	Inhibitor	99.79%	Unkown
HY-122594	BNS-22	Inhibitor	99.50%	Unkown
HY-W008874	meso-Tetra(4-pyridyl)porphine	Inhibitor	98.0%	Unkown
HY-118911	ATM Inhibitor-10	Inhibitor	98.7%	Unkown
HY-N0114A	(±)-Evodiamine		99.74%	Unkown
HY-123393	PNU-142586	Inhibitor	97.92%	Unkown
HY-13550	Ametantrone	Inhibitor	97.28%	Unkown
HY-160857	TOP5668	Agonist	98.07%	Unkown
HY-168489	OBI-992 drug-linker		96.64%	Unkown
HY-RS14902	TOPORS Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14894	TOP2A Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-126142	Top1 inhibitor 1	Inhibitor	99.67%	Unkown
HY-RS14893	TOP1MT Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14897	TOP2B Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14899	TOP3B Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-118942	Topovale	Inhibitor	99.94%	Unkown
HY-101115	PI3K-IN-6	Inhibitor	/	Unkown
HY-107133	Simmitecan hydrochloride		/	Unkown
HY-117142	Quizalofop-P	Inhibitor	/	Unkown
HY-117613	A-74932 hydrochloride	Inhibitor	/	Unkown
HY-120638	BMS-250749	Inhibitor	/	Unkown
HY-121560	L-leucyldoxorubicin		/	Unkown
HY-121866	CP-84364	Inhibitor	/	Unkown
HY-125387	TOP1210		/	Unkown
HY-125426	ToP-DNJ	Inhibitor	/	Unkown
HY-125500	TOP1362	Inhibitor	/	Unkown
HY-143265	Topoisomerase I inhibitor 2		/	Unkown
HY-144222	NSC 828467	Inhibitor	/	Unkown
HY-144654	HDAC/Top-IN-1	Inhibitor	/	Unkown
HY-145851	Top/HDAC-IN-1		/	Unkown
HY-145852	Top/HDAC-IN-2		/	Unkown
HY-146437	Topoisomerase I inhibitor 6	Inhibitor	/	Unkown
HY-149348	DiPT-4		/	Unkown
HY-159935	Top1-IN-2	Inhibitor	/	Unkown
HY-159966	Top/HDAC-IN-3		/	Unkown
HY-160856	TOP5300	Agonist	/	Unkown
HY-161957	Top1/2-IN-1	Inhibitor	/	Unkown
HY-163118	Topoisomerase II inhibitor 17		/	Unkown
HY-16560B	(R)-Camptothecin	Control	/	Unkown
HY-172259	Toyaburgine	Inhibitor	/	Unkown
HY-173001	AuL1	Inhibitor	/	Unkown
HY-175700	YCJ-02		/	Unkown
HY-175834	DNA/TOP2A-IN-1		/	Unkown
HY-176762	TOP3B-IN-1	Inhibitor	/	Unkown
HY-178029	RSK2/TOP2-IN-1		/	Unkown
HY-178036	ZM484	Inhibitor	/	Unkown
HY-180555	SN38-(PEG)2-Pom-α	Activator	/	Unkown
HY-181080	TOP1/TDP1-IN-1		/	Unkown
HY-18350A	Indimitecan hydrochloride	Inhibitor	/	Unkown
HY-184245	Hsp90-IN-48	Inhibitor	/	Unkown
HY-184984	SN-38-A2	Degrader	/	Unkown
HY-N15249	4"-Isovalerylspiramycin I		/	Unkown
HY-N17442	Echinoside A		/	Unkown
HY-P11715	Abz-GFDPFRQ-EDDnp		/	Unkown
HY-P4926	Mca-SEVKMDAEFRK(Dnp)RR-NH2		/	Unkown
HY-RS14890	TOP1 Human Pre-designed siRNA Set A		/	Unkown
HY-RS14891	Top1 Mouse Pre-designed siRNA Set A		/	Unkown
HY-RS14892	Top1 Rat Pre-designed siRNA Set A		/	Unkown
HY-RS14895	Top2a Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14896	Top2a Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14898	TOP3A Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-W1063697	5,8-Dimethoxyquinoxaline		/	Unkown
HY-W654323	PNU-142586 sodium	Inhibitor	/	Unkown
HY-W800535	Cryptolepine		/	Unkown
HY-107133A	Simmitecan		/	Unkown
HY-133219	Guajadial C	Inhibitor	/	Unkown
HY-169173	AL284	Inhibitor	/	Unkown
HY-180333	Top-Tdp tri inhibitor C12		/	Unkown
HY-184297	Topoisomerase II-IN-26	Inhibitor	/	Unkown
HY-185300	IKZF2-degrader 4	Degrader	/	Unkown
HY-RS20119	Top2b Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS21791	Top3a Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS26623	Top2b Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS28307	Top3a Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-W133968	Polyacrylate sodium		/	Unkown

Inhibitory Antibodies (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-P990947	Tilatamig	Inhibitor	99.99%	Unkown

Orthologs Information

Species	Symbol	Source	ID
Rattus norvegicus	TOPORS	RGD	RGD:1305270
Canis familiaris	TOPORS	VGNC	VGNC:47712
Macaca mulatta	TOPORS	VGNC	VGNC:79330
Mus musculus	TOPORS	MGD	MGI:2146189
Felis catus	TOPORS	VGNC	VGNC:66453
Bos taurus	TOPORS	VGNC	VGNC:36222
Others	TOPORS	NCBI