RANBP2 - RAN binding protein 2 Gene

Homo sapiens

Also known as ANE1; TRP1; TRP2; ADANE; IIAE3; NUP358

Gene ID: 5903 | Gene type: protein coding

About RANBP2

Cytogenetic location: 2q13 Genomic coordinates (GRCh38): 2:108,719,482-109,842,301 (from NCBI)

This gene has 19 transcripts (splice variants), 205 orthologues, 10 paralogues and is associated with 99 phenotypes. Ubiquitous expression in testis (RPKM 18.7), thyroid (RPKM 17.9) and 25 other tissues.

Summary

RAN is a small GTP-binding protein of the Ras superfamily that is associated with the nuclear membrane and is thought to control a variety of cellular functions through its interactions with Other proteins. This gene encodes a very large RAN-binding protein that immunolocalizes to the nuclear pore complex. The protein is a giant scaffold and mosaic cyclophilin-related nucleoporin implicated in the Ran-GTPase cycle. The encoded protein directly interacts with the E2 Enzyme UBC9 and strongly enhances SUMO1 transfer from UBC9 to the SUMO1 target SP100. These findings place sumoylation at the cytoplasmic filaments of the nuclear pore complex and suggest that, for some substrates, modification and nuclear import are linked events. This gene is partially duplicated in a gene cluster that lies in a hot spot for recombination on chromosome 2q. [provided by RefSeq, Jul 2008]

RANBP2 Products(1)

mRNA	Protein	Name
NM_006267.5	NP_006258.3	E3 SUMO-protein ligase RanBP2

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables SUMO ligase activity	IDA IDA: Inferred from direct assay	17264123	GOA
enables SUMO transferase activity	EXP EXP: Inferred from Experiment	11792325	GOA
NOT enables peptidyl-prolyl cis-trans isomerase activity	IDA IDA: Inferred from direct assay	20676357	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	10078529	GOA
enables small GTPase binding	IPI IPI: Inferred from physical interaction	7603572	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in NLS-bearing protein import into nucleus	IMP IMP: Inferred from mutant phenotype	7603572	GOA
involved in centrosome localization	IMP IMP: Inferred from mutant phenotype	20386726	GOA
involved in nuclear export	EXP EXP: Inferred from Experiment	27160050	GOA
involved in protein sumoylation	IDA IDA: Inferred from direct assay	22155184	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
part of SUMO ligase complex	IPI IPI: Inferred from physical interaction	22194619	GOA
located in annulate lamellae	IDA IDA: Inferred from direct assay	20386726	GOA
located in nuclear envelope	IDA IDA: Inferred from direct assay	20386726	GOA
located in nuclear inclusion body	IDA IDA: Inferred from direct assay	11839768	GOA
located in nuclear membrane	IDA IDA: Inferred from direct assay	11839768	GOA
part of nuclear pore	IDA IDA: Inferred from direct assay	17098863	GOA
part of nuclear pore cytoplasmic filaments	IDA IDA: Inferred from direct assay	7603572	GOA
part of nuclear pore nuclear basket	IDA IDA: Inferred from direct assay	11839768	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

RANBP2 Protein Structure

TPR_1

Ran_BP1

zf-RanBP

Ran_BP1

IR1-M

Ran_BP1

Pro_isomerase

0
500
1000
1500
2000
2500
3000
3224 a.a.

Protein Preferred Names

Protein Names

E3 SUMO-protein ligase RanBP2

358 kDa nucleoporin

RANBP2 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	RANBP2	P49792	BICD2	Homo sapiens	Q8TD16	Anti Bait CoIP	20386726
Intra	RANBP2	P49792	RANGAP1	Homo sapiens	P46060	Anti Tag CoIP	35271311
Intra	RANBP2	P49792	RANGAP1	Homo sapiens	P46060	Pull Down	16204249
Intra	RANBP2	P49792	ERBB2	Homo sapiens	P04626	Anti Bait CoIP	16314522
Intra	RANBP2	P49792	PRKN	Homo sapiens	O60260	IF	16332688
Intra	RANBP2	P49792	PRKN	Homo sapiens	O60260	Pull Down	16332688
Intra	RANBP2	P49792	PRKN	Homo sapiens	O60260	Anti Bait CoIP	16332688
Intra	RANBP2	P49792	PRKN	Homo sapiens	O60260	Anti Tag CoIP	16332688
Intra	RANBP2	P49792	PRKN	Homo sapiens	O60260	Y2H	16332688
Intra	RANBP2	P49792	C7orf25	Homo sapiens	Q9BPX7	FPS	37219487
Intra	RANBP2	P49792	UBE2I	Homo sapiens	P63279	Anti Tag CoIP	35271311
Intra	RANBP2	P49792	UBE2I	Homo sapiens	P63279	Y2H Pooling	20936779

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases

Alias

Familial Acute Necrotizing Encephalopathy

Adane

Recurrent Acute Necrotizing Encephalopathy

Hypohidrotic Ectodermal Dysplasia Autosomal Recessive

Anhidrotic Ectodermal Dysplasia, Autosomal Recessive	Autosomal Recessive Hypohidrotic Ectodermal Dysplasia
Ar-Hed	Autosomal Recessive Anhidrotic Ectodermal Dysplasia
Autosomal Recessive Hypohidrotic Ectodermal Dysplasia Syndrome	Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive

Ectodermal Dysplasia 10b, Hypohidrotic/Hair/Tooth Type, Autosomal Recessive

Hypohidrotic Ectodermal Dysplasia	Hed
Anhidrotic Ectodermal Dysplasia	Ectodermal Dysplasia, Hypohidrotic
Eda	Christ-Siemens-Touraine Syndrome
ECTD10B	Ectodermal Dysplasia Anhidrotic
Ectodermal Dysplasia, Anhidrotic	Cst Syndrome
Ectodermal Dysplasia Hypohidrotic Autosomal Recessive	Dysplasia, Ectodermal, Type 10b, Hypohidrotic/Hair/Tooth, Autosomal Recessive
Dysplasia, Ectodermal, Hypohidrotic	Ectodermal Dysplasia 11b, Hypohidrotic/Hair/Tooth Type, Autosomal Recessive
Ectodermal Dysplasia 3, Anhidrotic	Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive

Ectodermal Dysplasia 10a, Hypohidrotic/Hair/Nail Type, Autosomal Dominant

ECTD10A	Hed
Ectodermal Dysplasia 10a	Ectodermal Dysplasia Hypohidrotic Autosomal Dominant
Autosomal Dominant Hypohidrotic Ectodermal Dysplasia	Ectodermal Dysplasia, Hypohidrotic, Autosomal Dominant
Hypohidrotic Ectodermal Dysplasia Autosomal Dominant	Ad-Hed
Autosomal Dominant Anhidrotic Ectodermal Dysplasia	Ectodermal Dysplasia 3
Ed3	Eda3
Ectodermal Dysplasia, Anhidrotic, Autosomal Dominant	Dysplasia, Ectodermal, Type 10a, Hypohidrotic/Hair/Nail, Autosomal Dominant
Dysplasia, Ectodermal, Type 3

Mitochondrial Dna Depletion Syndrome 4a

Alpers Syndrome	Alpers-Huttenlocher Syndrome
Alpers Progressive Infantile Poliodystrophy	Alpers Diffuse Degeneration Of Cerebral Gray Matter With Hepatic Cirrhosis
Alpers Disease	Progressive Sclerosing Poliodystrophy
Pndc	Diffuse Cerebral Sclerosis Of Schilder
MTDPS4A	Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Alper'S Syndrome	Alpers' Disease Or Gray-Matter Degeneration
Diffuse Cerebral Degeneration In Infancy	Infantile Poliodystrophy
Poliodystrophia Cerebri Progressiva	Progressive Cerebral Poliodystrophy
Alpers' Disease	Alpers Progressive Sclerosing Poliodystrophy
Progressive Neuronal Degeneration Of Childhood With Liver Disease	Ahs
Mitochondrial Dna Depletion Syndrome 4a Alpers Type	Neuronal Degeneration Of Childhood With Liver Disease Progressive

Ectodermal Dysplasia

Congenital Ectodermal Defect	Congenital Ectodermal Dysplasia
Ectodermal Dysplasia Syndrome	Dysplasia, Ectodermal

Inflammatory Myofibroblastic Tumor

Inflammatory Fibrosarcoma

Mitochondrial Dna Depletion Syndrome 4b

Mitochondrial Neurogastrointestinal Encephalopathy Syndrome	Mngie Syndrome
Thymidine Phosphorylase Deficiency	MTDPS4B
Mitochondrial Neurogastrointestinal Encephalopathy Disease	Mngie
Myoneurogastrointestinal Encephalopathy Syndrome	Ogimd
Oculogastrointestinal Muscular Dystrophy	Polip
Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, And Intestinal Pseudo-Obstruction	Mitochondrial Neurogastrointestinal Encephalopathy Syndrome, Polg-Related
Mngie, Polg-Related	Mepop
Mitochondrial Myopathy With Sensorimotor Polyneuropathy, Ophthalmoplegia, And Pseudo-Obstruction	Mngie Disease
Mitochondrial Dna Depletion Syndrome 4b Mngie Type	Mitochondrial Neurogastrointestinal Encephalopathy Syndrome Polg-Related
Mngie Polg-Related	Mitochondrial Dna Depletion Syndrome, Type 4b
Visceral Myopathy Familial External Ophthalmoplegia

Tooth Agenesis

Oligodontia	Hypodontia
Selective Tooth Agenesis	Tooth Agenesis, Selective
Familial Tooth Agenesis	Anodontia
Congenital Absence Of One Tooth

Acute Necrotizing Encephalitis

Acute Necrotizing Encephalopathy	Postinfectious Acute Necrotizing Hemorrhagic Encephalopathy
Ane	Acute Necrotizing Encephalopathy Type 1
Adane	Ane1
Autosomal Dominant Acute Necrotizing Encephalopathy	Iiae3
Susceptibility To Acute Necrotizing Encephalopathy	Susceptibility To Infection-Induced Acute Encephalopathy
Encephalopathy, Acute Necrotizing, Susceptibility To	Encephalitis, Acute Necrotizing

Encephalopathy, Acute, Infection-Induced 4

Encephalopathy, Acute, Infection-Induced, Susceptibility To, 4	IIAE4
Encephalopathy, Acute, Infection-Induced, 4, Susceptibility To	Encephalopathy, Acute, Infection-Induced, 4
Acute Necrotizing Encephalopathy Of Childhood	Anec
Isolated Ane	Isolated Acute Necrotizing Encephalopathy
Encephalopathy Acute Infection-Induced 4

Ectodermal Dysplasia 11b, Hypohidrotic/Hair/Tooth Type, Autosomal Recessive

ECTD11B	Hed
Eda	Ectodermal Dysplasia 11b
Ectodermal Dysplasia, Hypohidrotic	Ectodermal Dysplasia, Anhidrotic
Ectodermal Dysplasia Anhidrotic	Ectodermal Dysplasia Hypohidrotic Autosomal Recessive
Dysplasia, Ectodermal, Type 11b, Hypohidrotic/Hair/Tooth, Autosomal Recessive	Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive

Epithelioid Inflammatory Myofibroblastic Sarcoma

Influenza

Flu	Influenza With Non-Respiratory Manifestation
Influenza With Other Manifestations	Influenza, Human
Influenza, Susceptibility To	Seasonal Influenza, Virus Identified

Encephalopathy

Brain Diseases	Encephalopathies
Toxic Encephalopathy	Toxic Brain Fever
Toxic Brain Inflammation	Toxic Brain Stem Inflammation
Toxic Cerebral Fever	Toxic Cerebrospinal Fever
Toxic Cerebrospinal Inflammation	Encephalopathy Nec
Encephalopathy Nos	Encephalopathy Disease
Encephalopathy Syndrome

Acute Hemorrhagic Encephalitis

8p11 Myeloproliferative Syndrome

8p11 Stem Cell Leukemia/Lymphoma Syndrome	8p11 Stem Cell Syndrome
Myeloid And Lymphoid Neoplasms With Fgfr1 Abnormalities	Stem Cell Leukemia/Lymphoma
Myeloid And Lymphoid Neoplasms With Fgfr1 Rearrangement	Chromosome 8p11 Myeloproliferative Syndrome

Chromosome 8p11 Myeloproliferative Syndrome

Stem Cell Leukemia/Lymphoma	Scll
Myeloid/Lymphoid Neoplasm Associated With Fgfr1 Rearrangement	8p11 Myeloproliferative Syndrome
Myeloid And Lymphoid Neoplasms With Fgfr1 Rearrangement

Fourth Cranial Nerve Palsy

Fourth Nerve Palsy	Fourth Cranial Nerve Paresis
Fourth Or Trochlear Nerve Palsy	4th Nerve Palsy
Fourth Cranial Nerve Paralysis	Superior Oblique Palsy
Trochlear Nerve Palsy	Trochlear Nerve Paralysis
Trochlear Nerve Weakness	Isolated Trochlear Nerve Palsy
Atrophy Of Fourth Cranial Nerve	Atrophy Of Trochlear Nerve

Pulmonary Plasma Cell Granuloma

Plasma Cell Granuloma, Pulmonary	Granuloma, Plasma Cell, Pulmonary
Lymphocytic Pseudotumor Of Lung	Sclerosing Hemangiocytoma Of Lung

Mesenchymal Cell Neoplasm

Benign Miscellaneous Mesenchymal Tumor	Mesenchymal Tumor
Mesenchymal Tumors

Postinfectious Encephalitis

Postinfective Encephalitis

Secondary Encephalitis

Lymphoblastic Lymphoma

Lymphoma, Lymphoblastic	Lymphoma Lymphoblastic
Precursor Cell Lymphoblastic Lymphoma	Precursor Cell Lymphoblastic Leukemia Lymphoma

Acute Disseminated Encephalomyelitis

Acute Disseminated Encephalitis	Adem
Ade	Encephalomyelitis Acute Disseminated
Encephalomyelitis, Acute Disseminated	Adem - [Acute Disseminated Encephalomyelitis]

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

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

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS11645	RANBP2 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	RANBP2	VGNC	VGNC:103649
Rattus norvegicus	RANBP2	RGD	RGD:1560047
Mus musculus	RANBP2	MGD	MGI:894323
Macaca mulatta	RANBP2	VGNC	VGNC:106205

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