1. Gene
  2. RANBP2 - RAN binding protein 2 Gene

RANBP2 - RAN binding protein 2 Gene

中文名称:RAN 结合蛋白 2

种属: Homo sapiens

同用名: ANE1; TRP1; TRP2; ADANE; IIAE3; NUP358

基因 ID: 5903 | 基因类型: protein coding

关于 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.

功能概要

RAN 是 Ras 超家族的一种小 GTP 结合蛋白,与核膜相关,被认为通过与其他蛋白的相互作用来控制多种细胞功能。该基因编码一种非常大的 RAN 结合蛋白,可免疫定位于核孔复合体。该蛋白质是一个巨大的支架和镶嵌亲环蛋白相关的核孔蛋白,与 Ran-GTPase 循环有关。编码的蛋白质直接与 E2 酶 UBC9 相互作用,并强烈增强 SUMO1 从 UBC9 转移到 SUMO1 目标 SP100。这些发现将 sumoylation 置于核孔复合体的细胞质细丝处,并表明,对于某些底物,修饰和核导入是相互关联的事件。该基因在基因簇中部分复制,该基因簇位于染色体 2q 上的重组热点。[RefSeq 提供,2008 年 7 月]

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 基因产物(1)

mRNA Protein Name
NM_006267.5 NP_006258.3 E3 SUMO-protein ligase RanBP2
基因本体论
  • 分子功能
  • 生物过程
  • 细胞组分
分子功能 GO 注释 逻辑证据 参考文献 来源
enables SUMO ligase activity IDA
IDA: 通过直接分析推断
17264123 GOA
enables SUMO transferase activity EXP
EXP: 通过实验结果推断
11792325 GOA
NOT enables peptidyl-prolyl cis-trans isomerase activity IDA
IDA: 通过直接分析推断
20676357 GOA
enables protein binding IPI
IPI: 通过物理相互作用推断
10078529 GOA
enables small GTPase binding IPI
IPI: 通过物理相互作用推断
7603572 GOA
生物过程 GO 注释 逻辑证据 参考文献 来源
involved in NLS-bearing protein import into nucleus IMP
IMP: 通过突变表型推断
7603572 GOA
involved in centrosome localization IMP
IMP: 通过突变表型推断
20386726 GOA
involved in nuclear export EXP
EXP: 通过实验结果推断
27160050 GOA
involved in protein sumoylation IDA
IDA: 通过直接分析推断
22155184 GOA
细胞组分 GO 注释 逻辑证据 参考文献 来源
part of SUMO ligase complex IPI
IPI: 通过物理相互作用推断
22194619 GOA
located in annulate lamellae IDA
IDA: 通过直接分析推断
20386726 GOA
located in nuclear envelope IDA
IDA: 通过直接分析推断
20386726 GOA
located in nuclear inclusion body IDA
IDA: 通过直接分析推断
11839768 GOA
located in nuclear membrane IDA
IDA: 通过直接分析推断
11839768 GOA
part of nuclear pore IDA
IDA: 通过直接分析推断
17098863 GOA
part of nuclear pore cytoplasmic filaments IDA
IDA: 通过直接分析推断
7603572 GOA
part of nuclear pore nuclear basket IDA
IDA: 通过直接分析推断
11839768 GOA
EXP:通过实验结果推断 IDA:通过直接分析推断 IPI:通过物理相互作用推断 IMP:通过突变表型推断 IGI:通过遗传相互作用推断 IEP:通过表达模式推断

RANBP2 蛋白结构

TPR_1

TPR_1: Tetratricopeptide repeat (60 - 92)

Ran_BP1

Ran_BP1: RanBP1 domain (1183 - 1303)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1351 - 1381)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1415 - 1443)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1479 - 1508)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1543 - 1571)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1606 - 1635)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1665 - 1694)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1724 - 1753)

zf-RanBP

zf-RanBP: Zn-finger in Ran binding protein and others (1781 - 1809)

Ran_BP1

Ran_BP1: RanBP1 domain (2024 - 2144)

Ran_BP1

Ran_BP1: RanBP1 domain (2321 - 2441)

IR1-M

IR1-M: Nup358/RanBP2 E3 ligase domain (2631 - 2694)

IR1-M

IR1-M: Nup358/RanBP2 E3 ligase domain (2709 - 2771)

Ran_BP1

Ran_BP1: RanBP1 domain (2922 - 3042)

Pro_isomerase

Pro_isomerase: Cyclophilin type peptidyl-prolyl cis-trans isomerase/CLD (3077 - 3222)

  • 0
  • 500
  • 1000
  • 1500
  • 2000
  • 2500
  • 3000
  • 3224 a.a.
蛋白主名 其他名称

E3 SUMO-protein ligase RanBP2

358 kDa nucleoporin

RANBP2 蛋白互作信息

分类
蛋白名称 蛋白编号 互作蛋白 互作蛋白种属 互作蛋白编号 实验方法 参考文献
种属内
RANBP2 P49792 BICD2 Homo sapiens Q8TD16 20386726
种属内
RANBP2 P49792 RANGAP1 Homo sapiens P46060 35271311
种属内
RANBP2 P49792 RANGAP1 Homo sapiens P46060 16204249
种属内
RANBP2 P49792 ERBB2 Homo sapiens P04626 16314522
种属内
RANBP2 P49792 PRKN Homo sapiens O60260
IF
16332688
种属内
RANBP2 P49792 PRKN Homo sapiens O60260 16332688
种属内
RANBP2 P49792 PRKN Homo sapiens O60260 16332688
种属内
RANBP2 P49792 PRKN Homo sapiens O60260 16332688
种属内
RANBP2 P49792 PRKN Homo sapiens O60260
Y2H
16332688
种属内
RANBP2 P49792 C7orf25 Homo sapiens Q9BPX7
FPS
37219487
种属内
RANBP2 P49792 UBE2I Homo sapiens P63279 35271311
种属内
RANBP2 P49792 UBE2I Homo sapiens P63279 20936779
种属间: 跨种属相互作用 种属内: 同种属相互作用

关联疾病

疾病名称 别名
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

疾病名称 别名
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

直系同源

种属 基因名 来源 基因 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