Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications

被引：35

作者：

McClure, Michelle L. ^{[1
]}

Barnes, Stephen ^{[2
]}

Brodsky, Jeffrey L. ^{[3
]}

Sorscher, Eric J. ^{[4
]}

机构：

[1] Univ Alabama Birmingham, Cyst Fibrosis Res Ctr, Birmingham, AL USA

[2] Univ Alabama Birmingham, Dept Pharmacol & Toxicol, Birmingham, AL USA

[3] Univ Pittsburgh, Dept Biol Sci, Pittsburgh, PA USA

[4] Emory Univ, Dept Pediat, 1760 Haygood Dr,Suite 280, Atlanta, GA 30322 USA

来源：

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY | 2016年 / 311卷 / 04期

基金：

美国国家卫生研究院;

关键词：

cystic fibrosis; protein trafficking; glycosylation; ubiquitination; phosphorylation; palmitoylation; ACTIVATED PROTEIN-KINASE; RETICULUM-ASSOCIATED DEGRADATION; AIRWAY EPITHELIAL-CELLS; ER-ASSOCIATED DEGRADATION; CYSTEINE-STRING PROTEIN; NUCLEOTIDE-BINDING DOMAINS; RECEPTOR DOWN-REGULATION; SPLEEN TYROSINE KINASE; CFTR CHLORIDE CHANNEL; ENDOPLASMIC-RETICULUM;

D O I：

10.1152/ajplung.00431.2015

中图分类号：

Q4 [生理学];

学科分类号：

071003 ;

摘要：

Posttranslational modifications add diversity to protein function. Throughout its life cycle, the cystic fibrosis transmembrane conductance regulator (CFTR) undergoes numerous covalent posttranslational modifications (PTMs), including glycosylation, ubiquitination, sumoylation, phosphorylation, and palmitoylation. These modifications regulate key steps during protein biogenesis, such as protein folding, trafficking, stability, function, and association with protein partners and therefore may serve as targets for therapeutic manipulation. More generally, an improved understanding of molecular mechanisms that underlie CFTR PTMs may suggest novel treatment strategies for CF and perhaps other protein conformational diseases. This review provides a comprehensive summary of co- and posttranslational CFTR modifications and their significance with regard to protein biogenesis.

引用

页码：L719 / L733

页数：15

共 209 条

[1] Global Cell Proteome Profiling, Phospho-signaling and Quantitative Proteomics for Identification of New Biomarkers in Acute Myeloid Leukemia Patients [J].

Aasebo, Elise ;

Forthun, Rakel B. ;

Berven, Frode ;

Selheim, Frode ;

Hernandez-Valladares, Maria .

CURRENT PHARMACEUTICAL BIOTECHNOLOGY, 2015, 17 (01) :52-70

[2] Palmitoylation and ubiquitination regulate exit of the Wnt signaling protein LRP6 from the endoplasmic reticulum [J].

Abrami, Laurence ;

Kunz, Beatrice ;

Lacovache, Ioan ;

Van der Goot, F. Gisou .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2008, 105 (14) :5384-5389

[3] Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation [J].

Accurso, Frank J. ;

Rowe, Steven M. ;

Clancy, J. P. ;

Boyle, Michael P. ;

Dunitz, Jordan M. ;

Durie, Peter R. ;

Sagel, Scott D. ;

Hornick, Douglas B. ;

Konstan, Michael W. ;

Donaldson, Scott H. ;

Moss, Richard B. ;

Pilewski, Joseph M. ;

Rubenstein, Ronald C. ;

Uluer, Ahmet Z. ;

Aitken, Moira L. ;

Freedman, Steven D. ;

Rose, Lynn M. ;

Mayer-Hamblett, Nicole ;

Dong, Qunming ;

Zha, Jiuhong ;

Stone, Anne J. ;

Olson, Eric R. ;

Ordonez, Claudia L. ;

Campbell, Preston W. ;

Ashlock, Melissa A. ;

Ramsey, Bonnie W. .

NEW ENGLAND JOURNAL OF MEDICINE, 2010, 363 (21) :1991-2003

[4] N-linked protein glycosylation in the ER [J].

Aebi, Markus .

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH, 2013, 1833 (11) :2430-2437

[5]

Ahmed T, 2016, CURR NEUROPHARMACOL

[6] Small heat-shock proteins select ΔF508-CFTR for endoplasmic reticulum-associated degradation [J].

Ahner, Annette ;

Nakatsukasa, Kunio ;

Zhang, Hui ;

Frizzell, Raymond A. ;

Brodsky, Jeffrey L. .

MOLECULAR BIOLOGY OF THE CELL, 2007, 18 (03) :806-814

[7] Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways [J].

Ahner, Annette ;

Gong, Xiaoyan ;

Frizzell, Raymond A. .

FEBS JOURNAL, 2013, 280 (18) :4430-4438

[8] Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway [J].

Ahner, Annette ;

Gong, Xiaoyan ;

Schmidt, Bela Z. ;

Peters, Kathryn W. ;

Rabeh, Wael M. ;

Thibodeau, Patrick H. ;

Lukacs, Gergely L. ;

Frizzell, Raymond A. .

MOLECULAR BIOLOGY OF THE CELL, 2013, 24 (02) :74-84

[9] Protein palmitoylation and subcellular trafficking [J].

Aicart-Ramos, Clara ;

Ana Valero, Ruth ;

Rodriguez-Crespo, Ignacio .

BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES, 2011, 1808 (12) :2981-2994

[10] Postranslational Modification of Ion Channels in Colonic Inflammation [J].

Akbarali, Hamid I. ;

Kang, Minho .

CURRENT NEUROPHARMACOLOGY, 2015, 13 (02) :234-238

← 1 2 3 4 5 6 7 8 9 10 →