Reactome | VHL [nucleoplasm]

VHL [nucleoplasm]

Stable Identifier

R-HSA-1234127

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

nucleoplasm

Locations in the PathwayBrowser

Expand all

Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - SUMOylation (Homo sapiens)
    - SUMO E3 ligases SUMOylate target proteins (Homo sapiens)
      - SUMOylation of ubiquitinylation proteins (Homo sapiens)
        
        PIAS4 SUMOylates VHL with SUMO1 (Homo sapiens)
        
        VHL [nucleoplasm] (Homo sapiens)

External Reference Information

External Reference

UniProt:P40337 VHL

Gene Names

VHL

Chain

chain:1-213

Reference Genes

BioGPS Gene:7428 VHL

COSMIC (genes):VHL VHL

CTD Gene:7428 VHL

dbSNP Gene:7428 VHL

ENSEMBL:ENSG00000134086 VHL

HGNC:12687 VHL

KEGG Gene (Homo sapiens):7428 VHL

Monarch:7428 VHL

NCBI Gene:7428 VHL

OMIM:608537 VHL

UCSC:P40337 VHL

Reference Transcript

Other Identifiers

0002680100

0003830215

11748633_a_at

11752180_x_at

1559227_s_at

1668_s_at

16937571

1LM8

1LQB

1VCB

203844_at

2610335

2610337

2610338

2610339

2610340

2610342

2610344

2610345

2610346

2610347

2610348

2610349

2610350

2610351

2610352

2610353

2610354

31631

31632

3ZRC

3ZRF

3ZTC

3ZTD

3ZUN

40871_at

4AJY

4AWJ

4B95

4B9K

4BKS

4BKT

4W9C

4W9D

4W9E

4W9F

4W9G

4W9H

4W9I

4W9J

4W9K

4W9L

4WQO

5430

5LLI

5N4W

5NVV

5NVW

5NVX

5NVY

5NVZ

5NW0

5NW1

5NW2

5T35

6BVB

6FMI

6FMJ

6FMK

6GFX

6GFY

6GFZ

6GMN

6GMQ

6GMR

6GMX

7428

8077779

8077781

A0A024R2F2

A0A0S2Z4K1

A_23_P132606

A_24_P134942

A_33_P3419938

AAA98614

AAB64200

AAH58831

AC034193

AF010238

AK315799

ALQ33844

ALQ33845

BAG38142

BC058831

CAA65343

CAB005430

CCDS2597

CCDS2598

CH471055

EAW64064

EAW64066

EAW64068

ENSG00000134086

ENSP00000256474

ENSP00000344757

ENST00000256474

ENST00000256474.2

ENST00000345392

ENST00000345392.2

EntrezGene:7428

EntrezGene:VHL

g4507890_3p_at

GE54104

GO:0000122

GO:0000902

GO:0003674

GO:0004842

GO:0005515

GO:0005575

GO:0005622

GO:0005623

GO:0005634

GO:0005654

GO:0005737

GO:0005739

GO:0005783

GO:0005829

GO:0006355

GO:0006464

GO:0006508

GO:0006950

GO:0007165

GO:0008134

GO:0008150

GO:0008219

GO:0008283

GO:0008285

GO:0009058

GO:0010629

GO:0016020

GO:0016567

GO:0019899

GO:0030154

GO:0030891

GO:0032991

GO:0034641

GO:0043066

GO:0043226

GO:0043687

GO:0045597

GO:0045893

GO:0046426

GO:0048856

GO:0050821

GO:0061418

GO:0061428

GO:0061630

HGNC:12687

HPA031631

HPA031632

Hs2.421597.1.A1_3p_s_at

ILMN_1738579

ILMN_1801984

ILMN_2376625

IPR002714

IPR022772

IPR024048

IPR024053

IPR036208

IPR037139

IPR037140

KU178386

KU178387

L15409

L15409_at

LRG_322

LRG_322t1

MIM:144700

MIM:171300

MIM:193300

MIM:263400

MIM:608537

NM_000551

NM_198156

NP_000542

NP_937799

PF01847

PF17211

PH_hs_0033268

TC03000055.hg

TC03002213.hg

U54612

UPI0000035C95

UPI00001E0587

VHL

VHL-201

VHL-202

X96489

Participant Of

input

PIAS4 SUMOylates VHL with SUMO1

hasComponent

VBC Complex [nucleoplasm]

Other forms of this molecule

SUMO1-K159,171,196-VHL [nucleoplasm]

VHL [cytosol]

Inferred To

Vhl [nucleoplasm] (Drosophila melanogaster)

Vhl [nucleoplasm] (Mus musculus)

Vhl [nucleoplasm] (Rattus norvegicus)

vhl [nucleoplasm] (Danio rerio)

vhl [nucleoplasm] (Xenopus tropicalis)

VHL [nucleoplasm] (Bos taurus)

VHL [nucleoplasm] (Gallus gallus)

VHL [nucleoplasm] (Sus scrofa)

vhl-1 [nucleoplasm] (Caenorhabditis elegans)

Cross References

RefSeq

NP_937799.1, NP_000542.1

OpenTargets

ENSG00000134086

GeneCards

P40337

ZINC - Substances

VHL_HUMAN

HPA

ENSG00000134086-VHL

ZINC target

P40337

Ensembl

ENSG00000134086, ENSP00000344757, ENSP00000256474, ENST00000256474, ENST00000345392

PRO

P40337

ZINC - Predictions - Purchasable

VHL_HUMAN

Orphanet

15708

PDB

6R7F, 4W9F, 4W9E, 6FMK, 5NVW, 6I7R, 4W9C, 6BVB, 6FMJ, 5NVV, 6I7Q, 6FMI, 5NW2, 5LLI, 4AWJ, 4W9J, 4WQO, 6R6H, 6GMN, 4W9I, 6HAY, 3ZUN, 4W9H, 6HAX, 4W9G, 6GFY, 5NVY, 6GMX, 1LM8, 5NVX, 6GFX, 1VCB, 4B95, 4W9D, 6GMR, 4W9L, 4W9K, 6GMQ, 3ZTD, 5NW1, 5T35, 5N4W, 4BKS, 3ZRC, 4B9K, 3ZTC, 5NW0, 4BKT, 1LQB, 5NVZ, 3ZRF, 6HR2, 6GFZ, 4AJY

Interactors (14)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:Q16665 HIF1A	7	2xHP-HIF1A [nucleoplasm] (R-HSA-1234105) HN-HIF1A [cytosol] (R-HSA-1234110) 2xHP-HIF1A [cytosol] (R-HSA-1234131) p-Y565,S797-HIF1A [cytosol] (R-HSA-9634700) p-Y565-HIF1A [cytosol] (R-HSA-8857584) HIF1A [cytosol] (R-HSA-1234102) HIF1A [nucleoplasm] (R-HSA-1234135)	0.957	20
UniProt:Q15370 ELOB	2	TCEB2 [nucleoplasm] (R-HSA-112423) ELOB [cytosol] (R-HSA-180551)	0.928	11
UniProt:Q13617 CUL2	3	CUL2 [cytosol] (R-HSA-416982) G76-NEDD8-K689-CUL2 [cytosol] (R-HSA-8952511) CUL2 [nucleoplasm] (R-HSA-1234126)	0.886	17
UniProt:Q15369 ELOC	2	TCEB1 [nucleoplasm] (R-HSA-112422) ELOC [cytosol] (R-HSA-180535)	0.784	7
UniProt:Q99750 MDFI			0.666	4
UniProt:P62877 RBX1	2	RBX1 [cytosol] (R-HSA-180582) RBX1 [nucleoplasm] (R-HSA-1234142)	0.623	4
UniProt:P63244 RACK1	1	GNB2L1 [cytosol] (R-HSA-5626956)	0.611	9
UniProt:P21980 TGM2			0.602	10
UniProt:Q99814 EPAS1	5	2xHP-EPAS1 [nucleoplasm] (R-HSA-1234151) HN-EPAS1 [cytosol] (R-HSA-1234112) 2xHP-EPAS1 [cytosol] (R-HSA-1234149) EPAS1 [cytosol] (R-HSA-1234119) EPAS1 [nucleoplasm] (R-HSA-452560)	0.547	2
UniProt:Q99873 PRMT1	2	PRMT1 [cytosol] (R-HSA-9632173) PRMT1 [nucleoplasm] (R-HSA-3215140)	0.508	2
UniProt:Q61221	7	Hif1a [nucleoplasm] (R-MMU-1234105) Hif1a [cytosol] (R-MMU-1234110) Hif1a [cytosol] (R-MMU-1234131) phospho-Hif1a [cytosol] (R-MMU-9634700) phospho-Hif1a [cytosol] (R-MMU-8857584) Hif1a [cytosol] (R-MMU-1234102) Hif1a [nucleoplasm] (R-MMU-1234135)	0.499	3
UniProt:P02751 FN1	8	FN1(32-271) [extracellular region] (R-HSA-3787935) FN1(32-2386) [platelet alpha granule lumen] (R-HSA-114620) FN1(272-2386) [extracellular region] (R-HSA-3788024) FN1(32-?) [extracellular region] (R-HSA-2533850) FN1(?-2386) [extracellular region] (R-HSA-2533841) p-FN1 [endoplasmic reticulum lumen] (R-HSA-8956937) FN1 [endoplasmic reticulum lumen] (R-HSA-8956708) FN1(32-2386) [extracellular region] (R-HSA-54851)	0.499	2
UniProt:Q05513 PRKCZ	5	p-T410,T563-PRKCZ [cytosol] (R-HSA-5218698) p-T410-PRKCZ [cytosol] (R-HSA-437181) PRKCZ [cytosol] (R-HSA-58218) PRKCZ [cell junction] (R-HSA-2134483) pS, p-T410-PRKCZ [cytosol] (R-HSA-9632824)	0.463	3
UniProt:P08151 GLI1	9	ub-pS-GLI1 [ciliary base] (R-HSA-5610514) ub-GLI1 [cytosol] (R-HSA-5635833) pS-GLI1 [ciliary base] (R-HSA-5610497) GLI1 [cytosol] (R-HSA-445457) GLI1 [ciliary tip] (R-HSA-5610360) GLI1 [ciliary base] (R-HSA-5610357) p-GLI1 [nucleoplasm] (R-HSA-5635042) 2p-GLI1 [nucleoplasm] (R-HSA-5635057) p-GLI1 [ciliary tip] (R-HSA-5635040)	0.463	2