Journal
FRONTIERS IN PHARMACOLOGY
Volume 9, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fphar.2018.01100
Keywords
peripheral QC; CFTR; stabilizer; ubiquitination; RFFL
Categories
Funding
- JSPS KAKENHI [JP25893275, 15H05643, 15H01192]
- Grants-in-Aid for Scientific Research [15H01192, 15H05643] Funding Source: KAKEN
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Conformationally defective cystic fibrosis transmembrane conductance regulator (CFTR) including rescued Delta F508-CFTR is rapidly eliminated from the plasma membrane (PM) even in the presence of a CFTR corrector and potentiator, limiting the therapeutic effort of the combination therapy. CFTR elimination from the PM is determined by the conformation-dependent ubiquitination as a part of the peripheral quality control (PQC) mechanism. Recently, the molecular machineries responsible for the CFTR PQC mechanism which includes molecular chaperones and ubiquitination enzymes have been revealed. This review summarizes the molecular mechanism of the CFTR PQC and discusses the possibility that the peripheral ubiquitination mechanism becomes a novel drug target to develop the CFTR stabilizer as a novel class of CFTR modulator.
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