USP7 - ubiquitin specific peptidase 7 Gene

Also Known as TEF1; HAUSP; HAFOUS

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 7874

About USP7

Cytogenetic location: 16p13.2 Genomic coordinates (GRCh38): 16:8,892,097-8,963,906 (from NCBI)

This gene has 23 transcripts (splice variants), 209 orthologues, 71 paralogues and is associated with 3 phenotypes. Ubiquitous expression in testis (RPKM 31.0), lymph node (RPKM 24.1) and 25 other tissues.

Summary

The protein encoded by this gene belongs to the peptidase C19 family, which includes ubiquitinyl hydrolases. This protein deubiquitinates target proteins such as p53 (a tumor suppressor protein) and WASH (essential for endosomal protein recycling), and regulates their activities by counteracting the opposing ubiquitin Ligase activity of proteins such as HDM2 and TRIM27, involved in the respective process. Mutations in this gene have been implicated in a neurodevelopmental disorder. [provided by RefSeq, Mar 2016]

USP7 Products (4)

mRNA	Protein	Name
NM_001286457.2	NP_001273386.2	ubiquitin carboxyl-terminal hydrolase 7 isoform 2
NM_001286458.2	NP_001273387.1	ubiquitin carboxyl-terminal hydrolase 7 isoform 3
NM_001321858.2	NP_001308787.1	ubiquitin carboxyl-terminal hydrolase 7 isoform 4
NM_003470.3	NP_003461.2	ubiquitin carboxyl-terminal hydrolase 7 isoform 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables K48-linked deubiquitinase activity	IDA IDA: Inferred from direct assay	25944111	GOA
enables cysteine-type deubiquitinase activity	EXP EXP: Inferred from Experiment	26235645	GOA
enables cysteine-type deubiquitinase activity	IDA IDA: Inferred from direct assay	16964248	GOA
enables cysteine-type deubiquitinase activity	IMP IMP: Inferred from mutant phenotype	11923872	GOA
enables cysteine-type endopeptidase activity	IMP IMP: Inferred from mutant phenotype	21745816	GOA
enables deubiquitinase activity	IMP IMP: Inferred from mutant phenotype	18410486	GOA
enables p53 binding	IDA IDA: Inferred from direct assay	20096447	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11279055	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in DNA alkylation repair	IDA IDA: Inferred from direct assay	25944111	GOA
acts upstream of or within monoubiquitinated protein deubiquitination	IDA IDA: Inferred from direct assay	26280536	GOA
involved in negative regulation of NF-kappaB transcription factor activity	IDA IDA: Inferred from direct assay	11279055	GOA
involved in negative regulation of TORC1 signaling	IDA IDA: Inferred from direct assay	35216969	GOA
involved in negative regulation of gene expression via chromosomal CpG island methylation	IMP IMP: Inferred from mutant phenotype	21745816	GOA
involved in negative regulation of gluconeogenesis	IDA IDA: Inferred from direct assay	28655758	GOA
involved in negative regulation of proteasomal ubiquitin-dependent protein catabolic process	IMP IMP: Inferred from mutant phenotype	26678539	GOA
involved in protein deubiquitination	IDA IDA: Inferred from direct assay	16964248	GOA
involved in protein deubiquitination	IMP IMP: Inferred from mutant phenotype	11923872	GOA
involved in protein stabilization	IDA IDA: Inferred from direct assay	21258371	GOA
involved in protein stabilization	IMP IMP: Inferred from mutant phenotype	18410486	GOA
involved in regulation of DNA-binding transcription factor activity	IDA IDA: Inferred from direct assay	16964248	GOA
involved in regulation of circadian rhythm	IDA IDA: Inferred from direct assay	27123980	GOA
involved in regulation of establishment of protein localization to telomere	IDA IDA: Inferred from direct assay	25172512	GOA
involved in regulation of protein stability	IDA IDA: Inferred from direct assay	27123980	GOA
involved in regulation of retrograde transport, endosome to Golgi	IMP IMP: Inferred from mutant phenotype	26365382	GOA
involved in symbiont-mediated disruption of host cell PML body	IDA IDA: Inferred from direct assay	20719947	GOA
involved in transcription-coupled nucleotide-excision repair	IMP IMP: Inferred from mutant phenotype	22466612	GOA

Cellular Component GO Annotation	Evidence	References	Source
is active in PML body	IDA IDA: Inferred from direct assay	20719947	GOA
located in cytosol	IDA IDA: Inferred from direct assay	11279055	GOA
located in nucleus	IDA IDA: Inferred from direct assay	11279055	GOA
part of protein-containing complex	IDA IDA: Inferred from direct assay	26678539	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

USP7 Protein Structure

MATH

UCH

USP7_ICP0_bdg

USP7_C2

0
200
400
600
800
1000
1102 a.a.

Protein Preferred Names	Protein Names
ubiquitin carboxyl-terminal hydrolase 7	Herpes virus-associated ubiquitin-specific protease

USP7 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	USP7	Q93009	CHEK2	Homo sapiens	O96017	FPS	39009827
Intra	USP7	Q93009	PCGF2	Homo sapiens	P35227	Anti Tag CoIP	20601937
Intra	USP7	Q93009	BMI1	Homo sapiens	P35226	Anti Tag CoIP	20601937
Cross	USP7	Q93009	Mdm4	Mus musculus	O35618	Anti Bait CoIP	20697359
Intra	USP7	Q93009	TSPYL5	Homo sapiens	Q86VY4	Anti Tag CoIP	21170034
Intra	USP7	Q93009	TSPYL5	Homo sapiens	Q86VY4	Anti Bait CoIP	21170034
Intra	USP7	Q93009	TSPYL5	Homo sapiens	Q86VY4	TAP	21170034
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	Anti Bait CoIP	16845383
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	Fluorescence Spectr	16474402
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	NMR	16474402
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	ITC	22056774
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	GMS	16402859
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	CoIP	11923872
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	Anti Bait CoIP	15916963
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	Pull Down	16474402
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	ITC	16402859
Intra	USP7	Q93009	TP53	Homo sapiens	P04637	X-Ray Diffraction	16474402
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	GMS	16402859
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	Pull Down	16474402
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	Fluorescence Spectr	16474402
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	Anti Bait CoIP	15916963
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	X-Ray Diffraction	16474402
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	Pull Down	15916963
Intra	USP7	Q93009	MDM2	Homo sapiens	Q00987	ITC	16402859
Intra	USP7	Q93009	MAL	Homo sapiens	P21145	Confocal	21988832
Intra	USP7	Q93009	MAL	Homo sapiens	P21145	Anti Tag CoIP	21988832
Intra	USP7	Q93009	MDM4	Homo sapiens	O15151	Anti Bait CoIP	15916963
Intra	USP7	Q93009	MDM4	Homo sapiens	O15151	Pull Down	15916963
Intra	USP7	Q93009	MDM4	Homo sapiens	O15151	Anti Bait CoIP	18566590
Intra	USP7	Q93009	RB1	Homo sapiens	P06400	IF	24823443
Intra	USP7	Q93009	RB1	Homo sapiens	P06400	Anti Bait CoIP	24823443
Intra	USP7	Q93009	RB1	Homo sapiens	P06400	Pull Down	24823443
Intra	USP7	Q93009	CBX8	Homo sapiens	Q9HC52	Anti Tag CoIP	20601937
Intra	USP7	Q93009	RNF2	Homo sapiens	Q99496	Anti Tag CoIP	20601937
Intra	USP7	Q93009	RING1	Homo sapiens	Q06587	Anti Tag CoIP	26496610
Intra	USP7	Q93009	RING1	Homo sapiens	Q06587	Anti Tag CoIP	20601937
Intra	USP7	Q93009	TBCB	Homo sapiens	Q99426	Pull Down	16713569
Intra	USP7	Q93009	DAXX	Homo sapiens	Q9UER7	Anti Bait CoIP	16845383
Intra	USP7	Q93009	DAXX	Homo sapiens	Q9UER7	Anti Bait CoIP	18566590
Intra	USP7	Q93009	DAXX	Homo sapiens	Q9UER7	Pull Down	16845383
Intra	USP7	Q93009	MAGEE1	Homo sapiens	Q9HCI5	Y2H	16713569
Intra	USP7	Q93009	MAGEE1	Homo sapiens	Q9HCI5	Phage Display	35044719
Intra	USP7	Q93009	BCOR	Homo sapiens	Q6W2J9	Y2H	16713569
Intra	USP7	Q93009	BCOR	Homo sapiens	Q6W2J9	Anti Tag CoIP	35271311
Intra	USP7	Q93009	BCOR	Homo sapiens	Q6W2J9	Anti Tag CoIP	26496610
Cross	USP7	Q93009	vIRF-4	Human herpesvirus 8	F5H9D8	GMS	22056774
Cross	USP7	Q93009	vIRF-4	Human herpesvirus 8	F5H9D8	ITC	22056774
Cross	USP7	Q93009	vIRF-4	Human herpesvirus 8	F5H9D8	Y2H	22056774
Cross	USP7	Q93009	vIRF-4	Human herpesvirus 8	F5H9D8	X-Ray Diffraction	22056774
Cross	USP7	Q93009	EBNA1	Epstein-Barr virus	P03211	NMR	16474402
Cross	USP7	Q93009	EBNA1	Epstein-Barr virus	P03211	GMS	16474402
Cross	USP7	Q93009	EBNA1	Epstein-Barr virus	P03211	Anti Tag CoIP	12783858
Cross	USP7	Q93009	EBNA1	Epstein-Barr virus	P03211	Fluorescence Spectr	16474402
Cross	USP7	Q93009	EBNA1	Epstein-Barr virus	P03211	Pull Down	16474402

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant USP7 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P74478	USP7 Protein, Human (sf9)	Q93009-1 (K208-E560)	≥ 90%, as determined by reducing SDS-PAGE.

USP7 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P80926	HAUSP/USP7 Antibody (YA657)	WB	Human
HY-P80926A	HAUSP/USP7 Antibody (YA657)(PBS only)	WB	Human
HY-P84715	HAUSP/USP7 Antibody (YA4412)	WB, IHC-P, ICC/IF, FC, ELISA	Human, Mouse, Rat, Monkey
HY-P84715A	HAUSP/USP7 Antibody (YA4412)(PBS only)	WB, IHC-P, ICC/IF, FC, ELISA	Human, Mouse, Rat, Monkey
HY-P85431	HAUSP/USP7 Antibody (YA5123)	WB, ICC/IF	Human, Mouse, Rat, Dog, Pig
HY-P85512	HAUSP/USP7 Antibody (YA5204)	WB	Human

Related Diseases

Diseases	Alias
Hao-Fountain Syndrome	HAFOUS Intellectual Developmental Disorder With Impaired Speech, Behavioral Abnormalities, And Dysmorphic Facies 16p13.2 Microdeletion Syndrome Del(16)(P13.2) Monosomy 16p13.2
Hypotonia
Fountain Syndrome	Mental Retardation, Sensorineural Deafness, Skeletal Abnormalities, And Coarse Face With Full Lips Deafness, Skeletal Dysplasia, Lip Granuloma Intellectual Disability, Deafness, Skeletal Abnormalities, Coarse Face With Full Lips Deafness-Skeletal Dysplasia-Coarse Face With Full Lips Syndrome Deafness-Skeletal Dysplasia-Lip Granuloma Syndrome Hearing Loss-Skeletal Dysplasia-Coarse Face With Full Lips Syndrome Hearing Loss-Skeletal Dysplasia-Lip Granuloma Syndrome
Uv-Sensitive Syndrome	Uvss Uv Sensitive Syndrome Ultraviolet Sensitive Syndrome
Cockayne Syndrome	Cockayne'S Syndrome Dwarfism-Retinal Atrophy-Deafness Syndrome Neill-Dingwall Syndrome Progeria-Like Syndrome Progeroid Nanism Cs
Schaaf-Yang Syndrome	Prader-Willi-Like Syndrome Chitayat-Hall Syndrome SHFYNG Pwls Magel2-Related Prader-Willi-Like Syndrome Magel2-Related Pwls Arthrogryposis, Distal, With Hypopituitarism, Mental Retardation, And Facial Anomalies Distal Arthrogryposis With Hypopituitarism, Intellectual Disability And Facial A Distal Arthrogryposis With Hypopituitarism, Intellectual Disability And Facial Anomalies Pws Due To A Point Mutation Pws Due To Point Mutation Prader-Willi Syndrome Due To A Point Mutation Prader-Willi Syndrome Due To Point Mutation Pws-Like
Prader-Willi Syndrome	Prader-Labhart-Willi Syndrome PWS Willi-Prader Syndrome Prader-Willi Syndrome Due To Translocation Prader-Willi Syndrome Due To Imprinting Mutation Prader-Willi Syndrome Due To Maternal Uniparental Disomy Of Chromosome 15 Prader Willi Syndrome Upd(15)Mat
Prostate Cancer	Prostate Carcinoma Prostate Cancer, Familial Prostate Neoplasm Prostate Cancer, Somatic Prostate Cancer, Susceptibility To Prostatic Cancer Prostatic Neoplasms Hereditary Prostate Cancer Prostatic Neoplasm Cancer Of Prostate Carcinoma Of Prostate Familial Prostate Cancer Familial Prostate Carcinoma Malignant Tumor Of Prostate Malignant Neoplasm Of Prostate Prostate Cancer, Familial, Susceptibility To Malignant Tumor Of The Prostate Ngp - New Growth Of Prostate Tumor Of The Prostate Prostate Cancer, Hereditary Cancer Of The Prostate Malignant Neoplasm Of The Prostate Prostatic Carcinoma PC Prca Cancer, Prostate Malignant Prostatic Tumour Malignant Tumour Of Prostate Primary Prostate Cancer Primary Malignant Neoplasm Of Prostate Prostate Gland Cancer
Encephalopathy, Neonatal Severe, Due To Mecp2 Mutations	Severe Congenital Encephalopathy Due To Mecp2 Mutation Severe Neonatal-Onset Encephalopathy With Microcephaly Encephalopathy, Neonatal Severe Neonatal Severe Encephalopathy Due To Mecp2 Mutations Mecp2-Related Severe Neonatal Encephalopathy Methyl-Cytosine Phosphate Guanine Binding Protein 2 Related Severe Neonatal Encephalopathy Severe Neonatal Encephalopathy Due To Mecp2 Mutations ENS-MECP2 Encephalopathy, Neonatal, Severe
Cockayne Syndrome A	Cockayne Syndrome Type 1 Cockayne Syndrome, Type A Cockayne Syndrome Type I CSA Cockayne Syndrome Classic Form Cockayne Syndrome Classical Cockayne Syndrome Type A Ckn1
Autism Spectrum Disorder	Asd Autism Spectrum Disorders Autistic Continuum Pervasive Developmental Disorder Pervasive Development Disorder Autistic Behavior Autistic Disorder Autistic Autistic Disorder Of Childhood Onset Infantile Autism Childhood Autism Kanner Syndrome Pervasive Developmental Delay Nos Pervasive Developmental Disorder, Not Otherwise Specified
Medulloblastoma	MDB Cpnet Localized Primitive Neuroectodermal Tumor Classic Medulloblastoma Medulloblastoma Predisposition Syndrome Medulloblastoma, Somatic Brain Medulloblastoma Cns Pnet Infratentorial Primitive Neuroectodermal Tumor Neuroectodermal Tumors, Primitive Medulloblastomas Desmoplastic Medulloblastoma Medulloblastoma, With Extensive Nodularity Medulloblastoma Of Unspecified Site Medullomyoblastoma Of Unspecified Site
Autosomal Dominant Cerebellar Ataxia	Spinocerebellar Ataxia Adca Pierre Marie Cerebellar Ataxia Ataxia, Spinocerebellar Sca Autosomal Dominant Spinocerebellar Ataxia Spinocerebellar Ataxias
Autism	Autistic Disorder Autism Susceptibility 1 Childhood Autism Autistic Disorder Of Childhood Onset Infantile Autism Kanner'S Syndrome Autistic

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

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (23)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-13453	BAY 11-7082	Inhibitor	99.98%	Unkown
HY-158039	YCH2823	Inhibitor	99.87%	Unkown
HY-101666	HBX 41108	Inhibitor	98.91%	Unkown
HY-136910	USP7-797	Inhibitor	99.09%	Unkown
HY-148369	U7D-1	Inhibitor	99.68%	Unkown
HY-175360	USP7 Ligand-Linker Conjugates 1	Inhibitor	99.92%	Unkown
HY-183282	LC-U7-44	Inhibitor	/	Unkown
HY-162794	YCH3124	Inhibitor	/	Unkown
HY-175359	USP7-IN-19	Inhibitor	/	Unkown
HY-180826	USP7 ligand-2	Ligand	/	Unkown
HY-153942	USP7-IN-12	Inhibitor	/	Unkown
HY-183689	OAT-4828	Inhibitor	/	Unkown
HY-159175	XM-U-14	Degrader	/	Unkown
HY-175358	PROTAC USP7 Degrader-1	Degrader	/	Unkown
HY-175793	USP7 ligand-1	Ligand	/	Unkown
HY-183078	P6620	Inhibitor	/	Unkown
HY-159964	USP7-IN-16	Inhibitor	/	Unkown
HY-49319	USP7-IN-14	Inhibitor	/	Unkown
HY-180793	PROTAC USP7 Degrader-2	Degrader	/	Unkown
HY-168121	USP7-IN-15	Inhibitor	/	Unkown
HY-124739	HBX 28258	Inhibitor	/	Unkown
HY-164544	USP7-055	Inhibitor	/	Unkown
HY-183942	USP7-IN-20	Inhibitor	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	USP7	VGNC	VGNC:54088
Rattus norvegicus	USP7	RGD	RGD:1306915
Felis catus	USP7	VGNC	VGNC:66898
Mus musculus	USP7	MGD	MGI:2182061
Macaca mulatta	USP7	VGNC	VGNC:99627
Bos taurus	USP7	VGNC	VGNC:36738
Others	USP7	NCBI