KEGG ORTHOLOGY: K03871

ORTHOLOGY: K03871

Entry

K03871                      KO

Symbol

VHL

Name

von Hippel-Lindau disease tumor supressor

Pathway

map04066

HIF-1 signaling pathway

map04120

Ubiquitin mediated proteolysis

map04212

Longevity regulating pathway - worm

map05200

Pathways in cancer

map05211

Renal cell carcinoma

Disease

H00021

Renal cell carcinoma

H00236

Congenital polycythemia

H00559

von Hippel-Lindau syndrome

H01510

Malignant paraganglioma

Brite

KEGG Orthology (KO) [BR:ko00001]
09120 Genetic Information Processing
  09123 Folding, sorting and degradation
   04120 Ubiquitin mediated proteolysis
    K03871  VHL; von Hippel-Lindau disease tumor supressor
09130 Environmental Information Processing
  09132 Signal transduction
   04066 HIF-1 signaling pathway
    K03871  VHL; von Hippel-Lindau disease tumor supressor
09150 Organismal Systems
  09149 Aging
   04212 Longevity regulating pathway - worm
    K03871  VHL; von Hippel-Lindau disease tumor supressor
09160 Human Diseases
  09161 Cancer: overview
   05200 Pathways in cancer
    K03871  VHL; von Hippel-Lindau disease tumor supressor
  09162 Cancer: specific types
   05211 Renal cell carcinoma
    K03871  VHL; von Hippel-Lindau disease tumor supressor
09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   04121 Ubiquitin system
    K03871  VHL; von Hippel-Lindau disease tumor supressor
Ubiquitin system [BR:ko04121]
Ubiquitin ligases (E3)
  Multi subunit Ring-finger type E3
   Cul2 complex
    Target recognizing subunit
     K03871  VHL; von Hippel-Lindau disease tumor supressor

Genes

HSA:

7428(VHL)

PTR:	749769(VHL)

PPS:	100986949(VHL)

GGO:	101149351(VHL)

PON:	100173372(VHL)

PPYG:

129033208(VHL)

NLE:	100600696(VHL)

HMH:	116466732(VHL)

SSYN:

129471655(VHL)

MCC:	106996285(VHL)

MCF:	107128974(VHL) 135970795(VHLL)

MTHB:

126947727 126952946

MNI:	105477690(VHL)

CSAB:

103223849(VHLL) 103227999(VHL)

CATY:

105585775(VHL)

PANU:

101024033(VHL)

TGE:	112618302(VHL) 112620309(VHLL)

MLEU:

105548680(VHLL) 105549541

RRO:	104667143(VHL)

RBB:	108512296(VHL) 108515508(VHLL)

TFN:	117074121(VHLL) 117083240(VHL)

PTEH:

111546933(VHL) 113221554(VHLL)

CANG:

105502134(VHLL) 105519078(VHL)

CJC:	103788323(VHL)

SBQ:	104651216(VHL)

CIMI:

108284664(VHL)

ANAN:

105730553(VHL)

MMUR:

105869051(VHL)

LCAT:

123623058(VHL)

PCOQ:

105812545(VHL)

OGA:	100952265(VHL)

MMU:	22346(Vhl)

MCAL:

110296136(Vhl)

MPAH:

110316548(Vhl)

RNO:	24874(Vhl)

MCOC:

116099407(Vhl)

ANU:	117715488(Vhl)

MUN:	110548945(Vhl)

CGE:	100751916(Vhl)

MAUA:

101826943(Vhl)

PROB:

127236149(Vhl)

PLEU:

114689616(Vhl) 119086770

MORG:

121441314(Vhl)

MFOT:

126505480

AAMP:

119807873(Vhl)

NGI:	103751433(Vhl)

HGL:	101697654(Vhl) 101705641

CPOC:

100729230(Vhl)

CCAN:

109688948(Vhl)

DORD:

105981299(Vhl)

DSP:	122108173(Vhl)

PLOP:

125358798(Vhl)

NCAR:

124970922 124989353 124993774

MMMA:

107150134(Vhl)

OCU:

100350053

OPI:	101531326(VHL)

TUP:	102486323(VHL)

GVR:	103607322(VHL)

CFA:	494000(VHL)

CLUD:

112661892(VHL)

VVP:	112933697(VHL)

VLG:	121496256(VHL)

NPO:	129522556(VHL)

AML:	100473201(VHL)

UMR:	103669642(VHL)

UAH:	113257162(VHL)

UAR:	123799204(VHL)

ELK:

111144039

LLV:

125108726

MPUF:

101679898(VHL)

MNP:	132011553(VHL)

MLK:	131824216(VHL)

NVS:	122903482 122909212(VHL)

ORO:	101370163(VHL)

EJU:	114207747(VHL)

ZCA:	113918488(VHL)

MLX:	118005246(VHL)

NSU:	110584820(VHL)

LWW:	102732787(VHL)

FCA:	101092590(VHL)

PYU:	121041270(VHL)

PCOO:

112858302(VHL)

PBG:	122487498(VHL)

PVIV:

125158887(VHL)

LRUF:

124524316

PTG:	102955568(VHL)

PPAD:

109254368(VHL)

PUC:

125929628

AJU:

106972341

HHV:	120231340(VHL)

BTA:	540957(VHL)

BOM:	102274048(VHL)

BIU:	109576248(VHL)

BBUB:

102411667(VHL)

BBIS:

104990148(VHL)

CHX:	102182921(VHL)

OAS:	101122897(VHL)

BTAX:

128050516(VHL)

ODA:	120867756(VHL)

CCAD:

122424663(VHL)

MBEZ:

129548072(VHL)

SSC:	100739306(VHL)

CBAI:

105067575(VHL)

CDK:	105093118(VHL)

VPC:	102543226(VHL)

BACU:

103007825(VHL)

BMUS:

118890565 118904300(VHL)

LVE:	103080565(VHL)

OOR:	101272871(VHL) 117200080

DLE:	111169977 111169978

PCAD:

102978007 102997043(VHL)

PSIU:

116749686 116762025(VHL)

NASI:

112412936(VHL)

ECB:	100058139(VHL)

EPZ:	103561463(VHL)

EAI:	106835626(VHL)

MYB:	102255400(VHL)

MLF:	102423240(VHL)

MDT:	132215401(VHL)

PKL:	118715204(VHL)

EFUS:

129147163(VHL)

MNA:	107534686(VHL)

DRO:	112301235(VHL)

SHON:

118993333 118993334(VHL)

AJM:	119038335 119041442 119058834

PDIC:

114501672(VHL) 114501724

PHAS:

123814422 123814423

MMF:	118633378(VHL)

PPAM:

129088403(VHL)

RFQ:	117037218(VHL)

PALE:

102884716

PGIG:

120621453(VHL)

PVP:	105299035(VHL)

RAY:	107498366(VHL)

TOD:	119255469(VHL)

SARA:

101538124(VHL)

SETR:

125998093(VHL)

LAV:	111750613(VHL)

TMU:

101352062

ETF:	101651152(VHL)

MDO:	100024305(VHL)

GAS:	123230700(VHL)

SHR:	111719447(VHL)

AFZ:

127551910

PCW:	110204865(VHL)

TVP:	118831402(VHL)

OAA:	114806529(VHL)

GGA:	416117(VHL)

PCOC:

116236614(VHL)

MGP:	109369884(VHL)

TPAI:

128078415

LMUT:

125698724(VHL)

NMEL:

110405041(VHL)

APLA:

110351418(VHL)

CATA:

118248073(VHL)

AFUL:

116493138(VHL)

TGU:	100231028(VHL)

LSR:	110477103(VHL)

SCAN:

115484105

PMOA:

120495629(VHL)

OTC:

121347398

PRUF:

121352382(VHL)

GFR:	102035931(VHL)

FAB:	101814622(VHL)

OMA:

130258101

PHI:	102106299(VHL)

PMAJ:

107210253(VHL)

CCAE:

111935274

CCW:

104683399

ETL:	114067683(VHL)

ZAB:

102062784

ZLE:	135453394(VHL)

ACHL:

103809450(VHL)

SVG:

106850383

MMEA:

130585471(VHL)

HRT:

120758473

SATI:

136366219

FPG:	101921920(VHL)

CCRI:

104155327(VHL)

SHAB:

115614319(VHL)

TALA:

116960445(VHL)

ACHC:

115353861(VHL)

HLE:	104835236(VHL)

AGEN:

126049962

GCL:

127021435

CSTI:

104555691

LDI:	104355006(VHL)

MNB:	103773750(VHL)

AVIT:

104280907(VHL)

EGZ:	104121781(VHL)

NNI:	104017014(VHL)

PCRI:

104024773

PADL:

103913610(VHL)

AFOR:

103902867

FGA:

104070157

GSTE:

104257232

CLV:	102086490(VHL)

MUI:	104544090(VHL)

PGUU:

104470926

PLET:

104614426

EHS:

104504933

CUCA:

104063633(VHL)

ACAR:

104518179

CVF:	104284545(VHL)

RTD:	128915571(VHL)

AAM:	106494625(VHL)

NPD:	112958721(VHL)

TGT:	104571463(VHL)

DNE:	112982921(VHL)

SCAM:

104148135(VHL)

ASN:	102378530(VHL)

AMJ:	102557707(VHL)

CPOO:

109311134(VHL)

GGN:	109288932(VHL)

PSS:	102455661(VHL)

CMY:	102935375(VHL)

CCAY:

125640136(VHL)

DCC:	119858615(VHL)

CPIC:

101937051(VHL)

TST:	117880653(VHL)

CABI:

116827058(VHL)

MRV:	120409383(VHL)

ACS:	100560920(vhl)

ASAO:

132769368(VHL)

PVT:	110077876(VHL)

SUND:

121922170(VHL)

PBI:

103056649

PMUR:

107298606(VHL)

CTIG:

120319518(VHL)

TSR:

106556309

PGUT:

117673909(VHL)

APRI:

131192334(VHL)

PTEX:

113451376(VHL)

NSS:	113419361(VHL)

PMUA:

114591793(VHL)

PRAF:

128408420(VHL)

ZVI:	118080790(VHL)

HCG:	128347830(VHL)

GJA:	107122229(VHL)

STOW:

125429934(VHL)

EMC:	129327473(VHL)

XLA:	108714636(vhl.L) 121393137

XTR:	549121(vhl)

NPR:	108786037(VHL)

BBUF:

120978599(VHL)

BGAR:

122943531(VHL)

GSH:	117351091(VHL)

DRE:	791202(vhl) 795635(vhll)

SRX:	107711035 107748400 107757273

SANH:

107667384 107673464 107694281

SGH:	107569299 107570200 107582681 107582682 107585106

CCAR:

109054466 109059967 109091426

CAUA:

113048358 113050402 113078230 113095019

CGIB:

127959564 127975939 128015779

PTET:

122342910(vhll) 122347199(vhl)

LROH:

127164408(vhll) 127167185(vhl)

OMC:	131539017(vhll) 131542131(vhl)

PPRM:

120476904(vhll) 120491502(vhl)

RKG:	130087516(vhl) 130089869(vhll)

MAMB:

125245314(vhll) 125273776(vhl)

CIDE:

127514035

CERY:

137011203(vhl) 137023878(vhll)

MASI:

127446319 127449315

TROS:

130547799(vhll) 130556707(vhl)

TDW:	130420271(vhll) 130425185(vhl)

MANU:

129431671(vhll) 129450551(vhl)

IPU:

108271809

IFU:	128614862(vhl)

PHYP:

113545808(vhl)

SMEO:

124387394(vhl)

TFD:	113640752(vhl)

TVC:	132844209(vhl)

TRN:	134317184(vhl)

AMEX:

103026553(vhl) 103029880(vhll)

CMAO:

118808307(vhl) 118812494(vhll)

EEE:	113572567 113575957

CHAR:

105897902(vhl)

TRU:

101068717

TFS:	130523816(vhl)

TNG:	GSTEN00027268G001

LCO:	104933452(vhl)

NCC:

104941408

TBEN:

117489895(vhl)

CGOB:

115008390(vhl)

PGEO:

117447257(vhl)

GACU:

117551024(vhl)

EMAC:

134883089(vhl)

ELY:	117256262(vhl)

EFO:	125893618(vhl)

PLEP:

121961549(vhl)

SLUC:

116041525 116041674

ECRA:

117943871(vhl)

ESP:	116688420(vhl)

PFLV:

114553605(vhl)

GAT:	120834864(vhl)

PPUG:

119194018(vhl)

AFB:	129106003(vhl)

CLUM:

117731291(vhl)

PSWI:

130208263(vhl)

MSAM:

119886535(vhl)

SCHU:

122866927(vhl)

CUD:	121506974(vhl)

ALAT:

119021920(vhl)

MZE:	101468402(vhl)

ONL:	100699102(vhl)

OAU:	116333841(vhl)

OLA:	101157853(vhl)

OML:	112145179(vhl)

CSAI:

133449457(vhl)

XMA:	102232438(vhl)

XCO:	114135786(vhl)

XHE:	116711000(vhl)

PRET:

PFOR:

PLAI:

PMEI:

GAF:	122834711(vhl)

PPRL:

129358387(vhl)

CVG:	107094015(vhl)

CTUL:

119776641(vhl)

GMU:	124856862(vhl)

NFU:

107385700

KMR:	108235772(vhl)

ALIM:

106518020(vhl)

NWH:	119412548(vhl)

AOCE:

111566652(vhl)

MCEP:

125007457(vhl)

CSEM:

103385940

POV:	109628898(vhl)

SSEN:

122768801(vhl)

HHIP:

117762308(vhl)

HSP:	118105248(vhl)

PPLT:

128425372(vhl)

SMAU:

118309860(vhl)

LCF:

108881483

SDU:	111221786(vhl)

SLAL:

111667871(vhl)

XGL:	120807547(vhl)

HCQ:	109522503(vhl)

SSCV:

125976897

SBIA:

133507889(vhl)

PEE:	133408590(vhl)

PTAO:

133483417(vhl)

BPEC:

110168396(vhl)

MALB:

109964425(vhl)

SJO:	128355614(vhl)

SASA:

100380306(vhl)

STRU:

115182051 115207849(vhl)

OTW:	112217436(vhl) 112239307(vhll)

OMY:	110494307(vhl) 110513649 110517247(vhll)

OGO:	124003432(vhl) 124020280 124027666 124028190

ONE:	115143161(vhl)

OKI:	109882540 109895633(vhl) 116360557

OKE:	118388580(vhl) 127927725(vhll)

SALP:

111966645(vhl)

SNH:	120025287(vhl) 120050156(vhll)

CCLU:

121555424 121575928(vhl) 123491260

ELS:	105017201(vhl) 105027074

SFM:	108920816 108923447

PKI:

111839852

AANG:

118211987(vhl)

LOC:	102682365(vhl) 102690813

PSPA:

121320092(vhll) 121329306(vhl)

ARUT:

117411739 117412333 117420124(vhll)

PSEX:

120539554(vhll) 120540336(vhl)

LCM:

102364581

CMK:	103176805(vhl) 103186103(vhll)

RTP:	109933532(vhll)

CPLA:

122559154(vhl) 122559723(vhll)

HOC:	132822167(vhl) 132824908(vhll)

LERI:

129704577(vhl)

LRJ:

BFO:

BBEL:

SPU:

LPIC:

APLC:

ARUN:

SKO:

DME:	Dmel_CG13221(Vhl)

DER:

6547113

DSE:

6608627

DSI:	Dsimw501_GD10785(Dsim_GD10785)

DYA:	Dyak_GE12873

DAN:

DSR:

DPO:

DPE:

DMN:	108159835 117187932

DWI:

6640477

DGR:

6560410

DMO:	Dmoj_GI19906

DAZ:

DNV:

DHE:

CCAT:

BOD:

BDR:

AOQ:

TDA:	119677001 119679081

MDE:	101892847 131801662

SCAC:

LCQ:

LSQ:

GFS:

ECOE:

CLON:

AGA:

ACOZ:

AARA:

AMER:

ASTE:

AFUN:

AMOU:

AAG:

AALB:

ASUA:

CQU:	CpipJ_CPIJ009992

CPII:

CNS:

BCOO:

ACER:

ADR:

AFLR:

BIM:

BBIF:

BVK:

BVAN:

BTER:

BAFF:

BPYO:

FVI:

CCAL:

OBB:

OLG:

MGEN:

CGIG:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

HST:

CFO:

FEX:

LHU:	105670331(Vhl)

PGC:

OBO:

VPS:

VCRB:

VVE:

CSOL:

TPRE:

LHT:

LBD:

MDL:

CGLO:

DAM:

AGIF:

VCAN:

CCIN:

DSM:

TCA:	103313576(Vhl) 657278

DPA:	109536001 109543800

SOY:

AGRG:

ATD:

CSET:

AGB:

LDC:

DVV:

NVL:	108566355 108566716

PPYR:

BMOR:

BMAN:

MSEX:

BANY:

MJU:

VCD:

MCIX:

PRAP:

LSIN:

AAGE:

HAW:

HZE:

TNL:

SLIU:

OFU:

ATRA:

GMW:

LGLY:

API:

DNX:

AGS:

RMD:

ACOO:

BTAB:

CLEC:

NLU:

PHU:	Phum_PHUM227970

ZNE:

110830843

CSEC:

111869957

BROR:

134530557

IEL:	124155282 124167102

FCD:

110846190

DPX:	DAPPUDRAFT_299820

DMK:

DPZ:

PVM:

PJA:

HAME:

CQD:

MNZ:

HAZT:

DSV:

RSAN:

RMP:

DPTE:

PTEP:

ABRU:

CEL:	CELE_F08G12.4(vhl-1)

CBR:	CBG_17370(Cbr-vhl-1)

CRQ:	GCK72_023767

NAI:	NECAME_08272

LOA:	LOAG_17040

HRO:	HELRODRAFT_194575

LGI:	LOTGIDRAFT_236999

PVUL:

126824154

PCAN:

112560760 112575448

BGT:

106066147

GAE:	121381853 121383346

HRF:	124120058 124138405

HRJ:	124278708 124288339

CRG:	105330196 117681582

CANU:

128155407 128180461

CVN:

111126589

OED:	125680617 125681124 130050344

MYI:	110441865 110447025

PMAX:

117321816 117336412

MMER:

123537222 123551740

RPHI:

132718065 132727680

DPOL:

127842889 127862395 127862405

MAEA:

128223047 128231312

OBI:

106868400

OSN:

115214222

LJP:	135468539 135480099

LAK:	106163863 106175781 106175783

LLON:

135501532

NVE:

116621125

EPA:	110254179 110254184

ATEN:

ADF:

AMIL:

PDAM:

SPIS:

XEN:

RES:

TAD:	TRIADDRAFT_7508

CME:	CYME_CMD063C

TBR:	Tb927.6.510

TBG:	TbgDal_VI140

XBC:	ELE36_17110

POJ:	PtoMrB4_48960

ACHR:

C2U31_27815

MROS:

EHO51_09545

MGRY:

MSR1_26150

LFS:	HFV01_04510

AVA:

Ava_3991

ANN:	EH233_03585

ROO:	G5S37_14980

» show all

Reference

PMID:18187417

Authors

Mahrour N, Redwine WB, Florens L, Swanson SK, Martin-Brown S, Bradford WD, Staehling-Hampton K, Washburn MP, Conaway RC, Conaway JW

Title

Characterization of Cullin-box sequences that direct recruitment of Cul2-Rbx1 and Cul5-Rbx2 modules to Elongin BC-based ubiquitin ligases.

Journal

J Biol Chem 283:8005-13 (2008)
DOI:10.1074/jbc.M706987200

Sequence

[hsa:7428]

Reference

PMID:15601820

Authors

Kamura T, Maenaka K, Kotoshiba S, Matsumoto M, Kohda D, Conaway RC, Conaway JW, Nakayama KI.

Title

VHL-box and SOCS-box domains determine binding specificity for Cul2-Rbx1 and Cul5-Rbx2 modules of ubiquitin ligases.

Journal

Genes Dev 18:3055-65 (2004)
DOI:10.1101/gad.1252404

Sequence

[hsa:7428]

Reference

PMID:10851083

Authors

Adryan B, Decker HJ, Papas TS, Hsu T

Title

Tracheal development and the von Hippel-Lindau tumor suppressor homolog in Drosophila.

Journal

Oncogene 19:2803-11 (2000)
DOI:10.1038/sj.onc.1203611

Sequence

[dme:Dmel_CG13221]

All links

Ontology (2)   
   KEGG BRITE (2)   
Pathway (10)   
   KEGG PATHWAY (10)   
Disease (4)   
   KEGG DISEASE (4)   
Gene (1026)   
   KEGG GENES (680)   
   KEGG MGENES (130)   
   RefGene (209)   
   OC (7)   
Literature (3)   
   PubMed (3)   
All databases (1045)   

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