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vhl-disease
VHL Disease
Hereditary VHL disease is caused by germline VHL mutations; clear cell RCC, CNS and retinal hemangioblastomas, pheochromocytoma (type 2), and pancreatic NETs; belzutifan (HIF-2α inhibitor) is FDA-approved for VHL-related tumors; type 1/2A/2B/2C classification by pheo risk.
Entry Metadata
| Field | Value |
|---|---|
| ID | vhl-disease |
| Name | VHL Disease |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toVhl
connects-toEgln1
connects-toRenal Cell Carcinoma
connects-toPheochromocytoma Paraganglioma
connects-toHif 1Alpha
connects-toBrain
connects-toEye
connects-toTuberous Sclerosis Complex
connects-toPancreas
connects-toErythropoietin
connects-toHlrcc
connects-toNeuroendocrine Tumors
connects-toEndothelial Cell
connects-toMen1 Syndrome
connects-toBirt Hogg Dube Syndrome
connects-toPolycythemia Vera
connects-toKidney
connects-toVegf
connects-toOxygen
connects-toAdrenal Gland
connects-toReproductive System
connects-toErythrocyte
connects-toEpas1
connects-toSdhb
connects-toIron
connects-toMtor
connects-toRegulatory T Cell
connects-toNatural Killer Cell
connects-toAkt
connects-toDendritic Cell
connects-toLiver
connects-toBone Marrow
connects-toNeuron
connects-toPdgf
connects-toPhoton
connects-toLung
connects-toAstrocyte
connects-toHemoglobin
connects-toElectron
connects-toGlucagon
connects-toAntibody
connects-toHypertension
connects-toNeutrophil
connects-toCyclin D1
connects-toT Cytotoxic Cell
connects-toStroke
connects-toMet
connects-toCkd
connects-toMast Cell
connects-toStat3
connects-toNf Kb
connects-toVenous Thromboembolism
connects-toEpilepsy
connects-toMajor Depressive Disorder
connects-toSepsis
connects-toHeart Failure
connects-toType 2 Diabetes
connects-toNeuropathic Pain
connects-toAnemia Of Chronic Disease
connects-toWound Healing
connects-toGeneralized Anxiety Disorder
connects-toNervous System
connects-toEndocrine System
connects-toDigestive System
connects-toCardiovascular System
connects-toRespiratory System
connects-toLymphatic System
connects-toTargeted Therapy
connects-toImmune System
connects-toNeurofibromatosis Type 2
connects-toIslet Of Langerhans
connects-toCancer Chemotherapy
connects-toGist
connects-toIdh Mutant Glioma
connects-toArterial Wall
connects-toMyeloproliferative Neoplasms
connects-toDiabetic Retinopathy
connects-toGlioblastoma
connects-toNeurofibromatosis Type 1
connects-toPeripheral Nerve
connects-toCortical Bone
connects-toMen4 Syndrome
connects-toMyc
connects-toEzh2
connects-toYap1
connects-toCdk4 6
connects-toTert
connects-toPik3Ca
connects-toCxcl12
connects-toSstr2
connects-toAdrenomedullin
connects-toFh
connects-toAngiopoietin
connects-toNorepinephrine
connects-toEgfr
connects-toBap1
connects-toNotch
connects-toE2F1
connects-toPten
connects-toErk1 2
connects-toIdh1
connects-toRb1
connects-toJak1 2
connects-toCgas Sting
connects-toStat1
connects-toGalectin 3
connects-toFoxo
connects-toSmad4
connects-toPerforin
connects-toGsk 3B
connects-toMdm2
connects-toS100A8 A9
connects-toSrc Kinase
connects-toDnmt3A
connects-toAutophagy
connects-toAmpk
connects-toCcr5
connects-toArid1A
connects-toIl 1B
connects-toIl 33
connects-toIl 6
connects-toIl 2
connects-toCalcium
connects-toTransferrin
connects-toMhc Class Ii
connects-toPd 1
connects-toCtla 4
connects-toNitric Oxide
connects-toIl 10
connects-toIron
connects-toIl 4
connects-toCholesterol
connects-toAdiponectin
connects-toIl 13
connects-toLeptin
connects-toResistin
connects-toHepcidin
connects-toType I Interferon
connects-toMacrophage
connects-toIfn Gamma
connects-toIl 12
connects-toIl 5
connects-toT Helper Cell
connects-toIl 23
connects-toIge
connects-toB Cell
connects-toComplement C5
connects-toC5Ar1
connects-toComplement C3
connects-toFactor H
connects-toC1 Esterase Inhibitor
connects-toTslp
connects-toBradykinin
connects-toPeriostin
connects-toHistamine
connects-toMelatonin
connects-toProlactin
connects-toTestosterone
connects-toSerotonin
connects-toOxytocin
connects-toVasopressin
connects-toSelenium
connects-toIodine
connects-toSodium
connects-toMagnesium
connects-toCopper
connects-toZinc
connects-toPotassium
connects-toPhosphorus
connects-toCarbon
connects-toChloride
connects-toHydrogen
connects-toNitrogen
connects-toSulfur
connects-toGlp 1
connects-toAngiotensin Ii
connects-toWnt Beta Catenin
connects-toRankl
connects-toSmad4
connects-toIl 2
connects-toVegf
connects-toFibronectin
connects-toIgf 1
connects-toActivin A
connects-toTgf Beta
connects-toCgrp
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toBdnf
connects-toOsteopontin