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cystic-fibrosis
Cystic Fibrosis
Cystic fibrosis is caused by biallelic CFTR mutations; defective chloride and bicarbonate transport → viscous mucus → progressive bronchiectasis, chronic Pseudomonas infection, and exocrine pancreatic insufficiency; median survival >40 years with CFTR modulator therapy.
Entry Metadata
| Field | Value |
|---|---|
| ID | cystic-fibrosis |
| Name | Cystic Fibrosis |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toCftr
connects-toPrss1
connects-toTgf Beta
connects-toNlrp3 Inflammasome
connects-toNeutrophil
connects-toIl 1B
connects-toHereditary Pancreatitis
connects-toChloride
connects-toLung
connects-toStaphylococcus Aureus
connects-toPancreas
connects-toCopd
connects-toType 2 Diabetes
connects-toRespiratory System
connects-toDigestive System
connects-toReproductive System
connects-toSodium
connects-toAspergillus Fumigatus
connects-toLiver
connects-toSmall Intestine
connects-toEndocrine System
connects-toOsteoporosis
connects-toFibrosis
connects-toMacrophage
connects-toImmune System
connects-toVitamin D
connects-toInsulin
connects-toMagnesium
connects-toNf Kb
connects-toT Helper Cell
connects-toOxygen
connects-toHydrogen
connects-toLarge Intestine
connects-toFibroblast
connects-toPhoton
connects-toSkin
connects-toIntestinal Epithelium
connects-toElectron
connects-toHeart
connects-toKidney
connects-toStomach
connects-toAntibody
connects-toErythrocyte
connects-toSpleen
connects-toOsteoclast
connects-toIron
connects-toType 1 Diabetes
connects-toIl 6
connects-toDendritic Cell
connects-toIl 17A
connects-toSepsis
connects-toMast Cell
connects-toColorectal Cancer
connects-toPulmonary Arterial Hypertension
connects-toStat3
connects-toCkd
connects-toPancreatic Cancer
connects-toMajor Depressive Disorder
connects-toVenous Thromboembolism
connects-toHcc
connects-toGeneralized Anxiety Disorder
connects-toIntegumentary System
connects-toHeart Failure
connects-toMusculoskeletal System
connects-toNervous System
connects-toCardiovascular System
connects-toRenal System
connects-toMycobacterium Tuberculosis
connects-toClostridioides Difficile
connects-toZinc Dietary
connects-toVancomycin
connects-toAmoxicillin
connects-toAsthma
connects-toTargeted Therapy
connects-toLung Slice
connects-toIslet Of Langerhans
connects-toHepatic Lobule
connects-toAnemia Of Chronic Disease
connects-toCortical Bone
connects-toAspergillus Fumigatus
connects-toInfluenza
connects-toAlveolus
connects-toTnf Alpha
connects-toHepatocyte
connects-toSurfactant
connects-toCcl2
connects-toHif 1Alpha
connects-toVegf
connects-toS100A8 A9
connects-toAutophagy
connects-toRankl
connects-toTlr4
connects-toEgfr
connects-toNitric Oxide
connects-toSecretory Iga
connects-toXanthine Oxidase
connects-toCaspase 3
connects-toNfe2L2
connects-toIl 10
connects-toComplement C3
connects-toMyd88
connects-toMtor
connects-toJak1 2
connects-toAkt
connects-toErk1 2
connects-toGalectin 3
connects-toStat1
connects-toCgas Sting
connects-toSmad4
connects-toFoxo
connects-toGsk 3B
connects-toPerforin
connects-toPik3Ca
connects-toAmpk
connects-toSrc Kinase
connects-toCcr5
connects-toDnmt3A
connects-toComplement C5
connects-toCxcl12
connects-toIl 33
connects-toCollagen
connects-toArid1A
connects-toCalcineurin
connects-toAdenosine
connects-toPotassium
connects-toCalcium
connects-toMhc Class Ii
connects-toProton
connects-toIl 13
connects-toTroponin Complex
connects-toProstaglandins
connects-toZinc
connects-toCholesterol
connects-toOmega 3 Fatty Acids
connects-toIl 4
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toHepcidin
connects-toLiver
connects-toSodium
connects-toOsteoporosis
connects-toAspergillus Fumigatus
connects-toType Ii Pneumocyte
connects-toType I Interferon
connects-toIl 5
connects-toIl 12
connects-toIl 23
connects-toC5Ar1
connects-toIge
connects-toAspergillus Fumigatus
connects-toFactor H
connects-toTransferrin
connects-toPeriostin
connects-toTslp
connects-toBradykinin
connects-toErythropoietin
connects-toHistamine
connects-toMelatonin
connects-toTestosterone
connects-toProlactin
connects-toOxytocin
connects-toVasopressin
connects-toSerotonin
connects-toSelenium
connects-toIodine
connects-toCopper
connects-toPhosphorus
connects-toSulfur
connects-toCarbon
connects-toNitrogen
connects-toAkt
connects-toPd 1
connects-toGlp 1
connects-toAngiotensin Ii
connects-toWnt Beta Catenin
connects-toIl 2
connects-toFibronectin
connects-toNotch
connects-toIgf 1
connects-toActivin A
connects-toCgrp
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toOsteopontin
connects-toFgfr
connects-toEpinephrine
Sources
- Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245(4922):1066-1073. · PubMed 2475911
- Heijerman HGM, McKone EF, Downey DG, et al. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation. Lancet. 2019;394(10212):1940-1948. · PubMed 31679946