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 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)

ASYL:

127679049(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)

ITI:	101956581(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

LGF:	123605966(VHL)

AJU:

106972341

PTG:	102955568(VHL)

PPAD:

109254368(VHL)

PUC:

125929628

PLEZ:

122203712(VHL)

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)

CSUM:

138087319(VHL)

ODA:	120867756(VHL)

CCAD:

122424663(VHL)

MREE:

136166625(VHL)

OVR:	110133520(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

LALB:

132527826(VHL) 132529859

DLE:	111169977 111169978

PCAD:

102978007 102997043(VHL)

PSIU:

116749686 116762025(VHL)

PPHC:

136129453(VHL)

NASI:

112412936(VHL)

ECB:	100058139(VHL)

EPZ:	103561463(VHL)

EAI:	106835626(VHL)

MYB:	102255400(VHL)

MLF:	102423240(VHL)

MDT:	132215401(VHL)

MYUM:

138992643(VHL)

PKL:	118715204(VHL)

EFUS:

129147163(VHL)

MNA:	107534686(VHL)

DRO:	112301235(VHL)

SHON:

118993333 118993334(VHL)

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)

SFUM:

130037436(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)

PBRV:

138158074(VHL)

OAA:	114806529(VHL)

TACU:

119950470(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)

ATRC:

139595722(VHL)

GFR:	102035931(VHL)

FAB:	101814622(VHL)

OMA:

130258101

PHI:	102106299(VHL)

PMAJ:

107210253(VHL)

CCAE:

111935274

CCW:

104683399

ACOE:

138117336

ETL:	114067683(VHL)

ZAB:

102062784

ZLE:	135453394(VHL)

ACHL:

103809450(VHL)

SVG:

106850383

MMEA:

130585471(VHL)

HRT:

120758473

SATI:

136366219

CCIC:

134048573(VHL)

FPG:	101921920(VHL)

CCRI:

104155327(VHL)

SHAB:

115614319(VHL)

TALA:

116960445(VHL)

ACHC:

115353861(VHL)

HLE:	104835236(VHL)

AGEN:

126049962

HHAR:

128136582(VHL)

GCL:

127021435

CSTI:

104555691

LDI:	104355006(VHL)

MNB:	103773750(VHL)

PPUS:

135182264(VHL)

AVIT:

104280907(VHL)

EGZ:	104121781(VHL)

NNI:	104017014(VHL)

PCRI:

104024773

PADL:

103913610(VHL)

AFOR:

103902867

FGA:

104070157

GSTE:

104257232

CBOY:

140657881(VHL)

CLV:	102086490(VHL)

CNIC:

135993049(VHL)

MUI:	104544090(VHL)

PGUU:

104470926

PLET:

104614426

EHS:

104504933

CUCA:

104063633(VHL)

ACAR:

104518179

CVF:	104284545(VHL)

RTD:	128915571(VHL)

CRID:

134521452(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)

ACAT:

141102760(VHL)

BBUF:

120978599(VHL)

BGAR:

122943531(VHL)

DTC:	141088781(VHL)

PWL:	138258971(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(vhll) 109066709 109091426(vhl)

CAUA:

113048358 113050402 113078230 113095019

CGIB:

127959564 127975939 128015779

PTET:

122342910(vhll) 122347199(vhl)

LROH:

127164408(vhll) 127167185(vhl)

GRF:	141333119(vhll) 141347412(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)

PDAB:

135746174(vhl) 135779147(vhll)

IPU:

108271809

IFU:	128614862(vhl)

PHYP:

113545808(vhl)

SMEO:

124387394(vhl)

TFD:	113640752(vhl)

TVC:	132844209(vhl)

CGAR:

128526792(vhl)

NGRA:

132885446(vhl)

TRN:	134317184(vhl)

AMEX:

103026553(vhl) 103029880(vhll)

CMAO:

118808307(vhl) 118812494(vhll)

EEE:	113572567 113575957

CHAR:

105897902(vhl)

SPIL:

134091821(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)

CANA:

137796518(vhl)

CVE:	137874068(vhl)

MSAM:

119886535(vhl)

SCHU:

122866927(vhl)

CUD:	121506974(vhl)

SPUL:

138606121(vhl)

LMIX:

132977745(vhl)

ALAT:

119021920(vhl)

ASTR:

137595519(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)

EJA:	138201227(vhl)

BFRE:

138626217(vhl)

MCEP:

125007457(vhl)

CSEM:

103385940

POV:	109628898(vhl)

SSEN:

122768801(vhl)

SSOE:

131461310(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(vhl)

SBIA:

133507889(vhl)

PEE:	133408590(vhl)

PTAO:

133483417(vhl)

BPEC:

110168396(vhl)

ENY:	140366064(vhl)

MALB:

109964425(vhl)

SJO:	128355614(vhl)

SSCO:

133977518(vhl)

GMH:

115557202

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)

OMM:	135515562 135515564 135532277 135539969(vhl)

OCLA:

139371068 139392867 139393200

SALP:

111966645(vhl)

SNH:	120025287(vhl) 120050156(vhll)

CCLU:

121555424 121575928(vhl) 123491260

ELS:	105017201(vhl) 105027074

OEP:	134029621(vhl)

SFM:	108920816 108923447

PKI:

111839852

AANG:

118211987(vhl)

AROT:

135238451(vhl)

CCOG:

133110414

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)

HAKA:

140734909(vhll) 140741933(vhl)

LRJ:

BFO:

BBEL:

SPU:

LPIC:

AJJ:

APLC:

ARUN:

AAMU:

AFIL:

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:

ALUD:

TDA:	119677001 119679081

MDE:	101892847 131801662

SCAC:

106092991

LCQ:

111682321

LSQ:

119616121

CVJ:	135961063(Vhl)

GFS:

ECOE:

CLON:

AGA:

ACOZ:

AARA:

AMER:

ASTE:

AMAP:

AFUN:

AMOU:

AMRS:

ACOS:

AAG:

AALB:

ASUA:

CQU:	CpipJ_CPIJ009992

CPII:

CBRI:

CNS:

BCOO:

LLL:

PPAP:

ACER:

ADR:

AFLR:

BIM:

BBIF:

BVK:

BVAN:

BTER:

BAFF:

BPYO:

FVI:

CCAL:

OBB:

OLG:

MGEN:

CGIG:

SOC:

MPHA:

AEC:

ACEP:

PBAR:

VEM:

TLN:

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

TMOL:

138122571 138123310(Vhl)

DPA:	109536001 109543800

ESIM:

EFOR:

SOY:

AGRG:

ATD:

CSET:

HAXR:

AGB:

LDC:

DVV:

NVL:	108566355 108566716

PPYR:

BMOR:

BMAN:

MSEX:

BANY:

MJU:

VCD:

MCIX:

PRAP:

LSIN:

AAGE:

HAW:

HZE:

TNL:

SLIU:

OFU:

ONU:

ATRA:

GMW:

LGLY:

CSTB:

CFUM:

141441840(Vhl)

API:

DNX:

AGS:

RMD:

ACOO:

BTAB:

CLEC:

NLU:

PHU:	Phum_PHUM227970

ZNE:

110830843

CSEC:

111869957

BROR:

134530557

IEL:	124155282 124167102

HAMR:

139692738(Vhl)

FCD:

110846190

DPX:	DAPPUDRAFT_299820

DMK:

DPZ:

AFRA:

PJA:

HAME:

MNZ:

MRJ:	136836486(galla-2)

HAZT:

DSV:

RSAN:

RMP:

GOE:

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:

PCAN:

LSAX:

BGT:

GAE:	121381853 121383346

HRF:	124120058 124138405

HRJ:	124278708 124288339

HASI:

HCRA:

CRG:

CANU:

CVN:

OED:	125680617 125681124 130050344

MYI:	110441865 110447025

PMAX:

AIRR:

MMER:

RPHI:

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:

MCAZ:

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

ROO:	G5S37_14980

LFS:	HFV01_04510

AVA:

Ava_3991

ANN:	EH233_03585

» 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]

LinkDB

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