Nitric oxide metabolism in cystic fibrosis (WP4947)

Homo sapiens

This pathway describes the NO metabolism in cystic fibrosis (CF) and is based on Figure 1 of Brinkmann et al. 2020.


Friederike Ehrhart and Eric Weitz


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Homo sapiens


Rare Diseases


Pathway Ontology

disease pathway nitric oxide mediated signaling pathway

Disease Ontology

cystic fibrosis


Label Type Compact URI Comment
L-Arginine Metabolite chebi:16467
Nω,Nω- dimethyl- L- arginine Metabolite chebi:17929 ADMA
L-arginine residue Metabolite chebi:29952
Nitric oxide Metabolite chebi:16480
Nitrogen dioxide Metabolite chebi:33101
Nitrooxidanyl Metabolite chebi:29329
Dimethylamine Metabolite chebi:17170 DMA
Nω,N'ω-dimethyl-L-arginine Metabolite chebi:25682 SDMA
Nω,Nω-dimethyl-L-arginine residue Metabolite chebi:61896
NOS1 GeneProduct ensembl:ENSG00000089250
DDAH1 GeneProduct ncbigene:23576
DDAH2 GeneProduct ncbigene:23564
PRMT1 GeneProduct ensembl:ENSG00000126457
PRMT2 GeneProduct ensembl:ENSG00000160310
PRMT3 GeneProduct ensembl:ENSG00000185238
CARM1 GeneProduct ensembl:ENSG00000142453
PRMT5 GeneProduct ensembl:ENSG00000100462
PRMT6 GeneProduct ensembl:ENSG00000198890
PRMT7 GeneProduct ensembl:ENSG00000132600
PRMT8 GeneProduct ensembl:ENSG00000111218
Carboxyl anhydrase GeneProduct pfam:PF00194
NOS3 GeneProduct ensembl:ENSG00000164867
NOS2 GeneProduct ensembl:ENSG00000007171


  1. Transport and interaction of nitrogen oxides and NO2 with CO2-HCO3- transporters in pancreatic acini. Zhao H, Xu X, Ujiie K, Star RA, Muallem S. Am J Physiol. 1994 Aug;267(2 Pt 1):C385-93. PubMed Europe PMC Scholia
  2. Activated L-Arginine/Nitric Oxide Pathway in Pediatric Cystic Fibrosis and Its Association with Pancreatic Insufficiency, Liver Involvement and Nourishment: An Overview and New Results. Brinkmann F, Hanusch B, Ballmann M, Mayorandan S, Bollenbach A, Chobanyan-Jürgens K, et al. J Clin Med. 2020 Jun 26;9(6):2012. PubMed Europe PMC Scholia