Related Experiment Video

Updated: Mar 16, 2026

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

Published on: May 10, 2014

c- Src and its role in cystic fibrosis

María Macarena Massip Copiz¹, Tomás Antonio Santa Coloma¹

¹Institute for Biomedical Research (BIOMED), Laboratory of Cellular and Molecular Biology, National Scientific and Technical Research Council (CONICET) and School of Medical Sciences, Pontifical Catholic University of Argentina (UCA), Buenos Aires, Argentina.

European Journal of Cell Biology

|August 18, 2016

Abstract:

Cystic fibrosis (CF) is a lethal inherited disease produced by mutations in the gene encoding the CFTR chloride channel. Loss of function in the CFTR gene is associated with a not much noticed increased expression and activity of the non-receptor protein-tyrosine kinase c-Src. CF is therefore the result from the loss of CFTR chloride transport function and its consequences, including a chronic and excessive c-Src signaling. On the other hand, c-Src, encoded by the SRC gene, is involved in diverse signaling mechanisms that regulate key cellular functions such as cell proliferation, apoptosis, oxidative stress, inflammation, and innate immunity. These c-Src-regulated cellular functions are also affected in CF; however, studies exploring a direct role of c-Src in the regulation of these cellular functions in CF are yet scarce and often controversial. Here we describe the c-Src regulation and functions, with emphasis in those altered in CF, and describe the role of CFTR as a "signaling molecule" that negatively modulates c-Src expression and activity. It is also discussed the emerging role of intracellular Cl^- and IL-1β as intermediate signaling effectors between CFTR and c-Src.

Insights

Cystic fibrosis (CF) results from CFTR gene mutations, leading to excessive c-Src signaling. This study explores CFTR

Area of Science:

Molecular Biology
Cellular Signaling
Genetics

Background:

Cystic Fibrosis (CF) is a genetic disorder caused by CFTR gene mutations.
CFTR dysfunction is linked to increased non-receptor protein-tyrosine kinase c-Src activity.
c-Src regulates crucial cellular functions like proliferation, apoptosis, and immunity, which are altered in CF.

Purpose of the Study:

To elucidate c-Src regulation and functions in CF.
To investigate the role of CFTR as a negative modulator of c-Src.
To explore the involvement of intracellular Cl- and IL-1β in CFTR-c-Src signaling.

Main Methods:

Literature review and analysis of existing studies on CFTR and c-Src.
Discussion of signaling pathways involving c-Src in cellular processes.
Examination of the interplay between CFTR, c-Src, intracellular Cl-, and IL-1β.

Main Results:

CFTR loss of function leads to chronic and excessive c-Src signaling.
CFTR acts as a negative regulator of c-Src expression and activity.
Intracellular Cl- and IL-1β emerge as potential signaling intermediates.

Conclusions:

CFTR is a critical modulator of c-Src signaling, impacting cellular functions in CF.
Understanding the CFTR-c-Src axis offers potential therapeutic targets for CF.
Further research is needed to confirm the roles of intracellular Cl- and IL-1β.

Read full analysis

Keywords:

CFTR Cystic fibrosis IL-1β IL1RN Intracellular chloride PP2 c-Src

More Related Videos

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein

Published on: March 9, 2015

Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein in Saccharomyces cerevisiae

Expression and Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein in Saccharomyces cerevisiae

Published on: March 10, 2012

Related Concept Videos

Cystic Fibrosis: Pathogenesis

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...

Cystic Fibrosis: Management

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...

GPCRs Regulate Adenylyl Cylase Activity

GPCRs Regulate Adenylyl Cylase Activity

Some GPCRs transmit signals through adenylyl cyclase (AC), a transmembrane enzyme. AC helps synthesize second messenger cyclic adenosine monophosphate (cAMP). AC catalyzes cyclization reaction and converts ATP to cAMP by releasing a pyrophosphate. The pyrophosphate is further hydrolyzed to phosphate by the enzyme pyrophosphatase, which drives cAMP synthesis to completion. However, cAMP is rapidly degraded to 5′ AMP by the enzymes phosphodiesterase (PDE), preventing overstimulation of...

Transport Across the Golgi

Transport Across the Golgi

While it is unclear how molecules move between adjacent Golgi cisternae, it is apparent that the molecules move from cis- cisterna, the entry face, to the trans- cisterna, the exit face. Experiments initially suggested vesicles that bud from one cisterna and fuse with the next cisterna to transport proteins between the cisternae. This vesicular transport model describes the Golgi apparatus as a relatively static structure with a unique enzyme composition in each cisterna. Molecules are...

Cotranslational Protein Translocation

Cotranslational Protein Translocation

Translocation of proteins across membranes is an ancient process that occurs even in bacteria and archaebacteria. In fact, the components of the translocation machinery are still conserved between prokaryotes and eukaryotes.
Sec61 channel partners for cotranslational translocation
During cotranslational translocation, the Sec61 channel partners with the signal recognition particle (SRP), the signal recognition particle receptor (SR), and the ribosomes to transport the nascent polypeptide chain...

Rab Cascades

Rab Cascades

Rab GTPases act in a regulated cascade during membrane fusion, helping the lipid bilayers mix. The Rab family of proteins are active when bound to GTP, and inactive when bound to GDP. Hence, they act as guanine nucleotide-dependent molecular switches. Rab-GTP recognizes and binds to long or short-range tethering proteins to capture the target vesicle. These tethers coordinate with SNAREs on the vesicle and the target membrane to assemble the trans SNARE complex that locks the mixing bilayers.

Articles linked to this work by shared authors, journal, and citation graph.

Folic Acid and Endothelial Dysfunction in COVID-19.

Life (Basel, Switzerland)·2026

See all related articles