STANDARD_NAME	BIOCARTA_CFTR_PATHWAY
SYSTEMATIC_NAME	M12399
COLLECTION	C2:CP:BIOCARTA
MSIGDB_URL	https://www.gsea-msigdb.org/gsea/msigdb/human/geneset/BIOCARTA_CFTR_PATHWAY
NAMESPACE	HUMAN_SEQ_ACCESSION
DESCRIPTION_BRIEF	Cystic fibrosis transmembrane conductance regulator (CFTR) and beta 2 adrenergic receptor (b2AR) pathway
DESCRIPTION_FULL	The defects in cAMP-regulated chloride channel CTFR are believed to be the major cause for cystic fibrosis. Regulation of CFTR protein by the surface receptor beta adrenergic receptor is mediated through the ezrin/radixin/moesin binding phosphoprotein 50 (EBP50), which binds both the C-termini CFTR and b2AR through their PDZ binding motifs. In the resting state, CFTR, b2AR, and EBP50 exist as a macromolecular complex on the apical surface of epithelial cells. Upon agonist activation of the b2AR, the adenulate cyclase is stimulated through the G protein pathway, leading to an increase in cAMP. The elevated concentration of cAMP activates PKA, which is anchored near CFTR via interaction with Ezrin. The phosphorylation of CFTR by PKA disrupts the complex and leads to compartmentalized and specific signaling of the channel.
PMID	
GEOID	
AUTHORS	
CONTRIBUTOR	BioCarta
CONTRIBUTOR_ORG	BioCarta
EXACT_SOURCE	
FILTERED_BY_SIMILARITY	
EXTERNAL_NAMES_FOR_SIMILAR_TERMS	
EXTERNAL_DETAILS_URL	https://data.broadinstitute.org/gsea-msigdb/msigdb/biocarta/human/h_cftrPathway.gif
SOURCE_MEMBERS	AK094467,NM_000024,NM_000492,NM_001111077,NM_001164758,NM_001164759,NM_001164760,NM_001164761,NM_001164762,NM_001242857,NM_001242858,NM_001242859,NM_001242860,NM_001242861,NM_001242862,NM_002731,NM_002732,NM_002734,NM_002735,NM_002736,NM_003379,NM_004157,NM_004252,NM_021116,NM_182948,NM_207578,NM_212471,NM_212472,S82430,X73053
GENE_SYMBOLS	NHERF1,ADRB2,CFTR,EZR,PRKAR1B,PRKAR1B,PRKAR1B,PRKAR1B,PRKAR1B,PRKACB,PRKACB,PRKACB,PRKACB,PRKACB,PRKACB,PRKACB,PRKACG,PRKAR1A,PRKAR1B,PRKAR2B,EZR,PRKAR2A,NHERF1,ADCY1,PRKACB,PRKACB,PRKAR1A,PRKAR1A,CFTR,CFTR
FOUNDER_NAMES