@@ -88,14 +88,14 @@ export function Description() {
<p>Although there is currently no cure for CF, patients must manage the disease throughout their lives, relying on treatments that alleviate symptoms but do not address the root cause. This lifelong management imposes significant financial burdens on affected families and healthcare systems, particularly in regions with a high prevalence of CF <SupScrollLinklabel="11"/>{/*ehem15*/} . In recent years, <b>CFTR modulators</b>, which target the underlying genetic defect, have offered new hope for many patients. </p>
<p>The CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein is an ion channel that facilitates the movement of chloride ions across epithelial cell membranes<SupScrollLinklabel="17"/><SupScrollLinklabel="18"/> . This movement is essential for controlling the flow of water in tissues such as the lungs and intestines <SupScrollLinklabel="19"/> . This increase in ion concentration in the extracellular space draws water out of the cells and into the surrounding mucus or fluid, ensuring it stays thin and mobile <SupScrollLinklabel="20"/> .</p>
<p>The CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) protein is an ion channel that facilitates the movement of chloride ions across epithelial cell membranes<SupScrollLinklabel="18"/> . This movement is essential for controlling the flow of water in tissues such as the lungs and intestines <SupScrollLinklabel="19"/> . This increase in ion concentration in the extracellular space draws water out of the cells and into the surrounding mucus or fluid, ensuring it stays thin and mobile <SupScrollLinklabel="20"/> .</p>
<p>The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is a specialized protein that plays a crucial role in maintaining the balance of ions and water on the surface of certain cells, particularly in the lungs, pancreas, and other organs <SupScrollLinklabel="18"/><SupScrollLinklabel="21"/> . </p>
<H4text="Structure of CFTR"id="structure-cftr"/>
<p>CFTR is a large protein embedded in the cell membrane. It belongs to a family of proteins known as ABC transporters (ATP-Binding Cassette transporters), which typically move molecules across cell membranes<SupScrollLinklabel="17"/>. CFTR, however, is unique because it functions as an ion channel rather than a transporter <SupScrollLinklabel="18"/> . </p>
<p>CFTR is a large protein embedded in the cell membrane. It belongs to a family of proteins known as ABC transporters (ATP-Binding Cassette transporters), which typically move molecules across cell membranes. CFTR, however, is unique because it functions as an ion channel rather than a transporter <SupScrollLinklabel="18"/> . </p>
<p>The protein consists of several important regions: </p>
<ul>
<li><b>Two transmembrane domains (TMDs)</b>: These span the cell membrane and create the channel through which ions can flow <SupScrollLinklabel="20"/> .</li>
<li><b>Two nucleotide-binding domains (NBDs)</b>: Located on the cytoplasmic side of the membrane, these domains bind and hydrolyze ATP (adenosine triphosphate). ATP binding and hydrolysis regulate the opening and closing of the chloride channel<SupScrollLinklabel="17"/><SupScrollLinklabel="21"/> .</li>
<li><b>Two nucleotide-binding domains (NBDs)</b>: Located on the cytoplasmic side of the membrane, these domains bind and hydrolyze ATP (adenosine triphosphate). ATP binding and hydrolysis regulate the opening and closing of the chloride channel<SupScrollLinklabel="21"/> .</li>
<li><b>Regulatory (R) domain</b>: This domain is unique to CFTR and controls the activity of the protein. It requires phosphorylation by protein kinase A (PKA) to activate the ion channel <SupScrollLinklabel="18"/><SupScrollLinklabel="19"/> .</li>
</ul>
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@@ -110,12 +110,12 @@ export function Description() {
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<H4text="Function of CFTR"id="function-cftr"/>
<p>CFTR functions primarily as a chloride ion channel. It is responsible for transporting chloride ions (Cl⁻) across epithelial cell membranes<SupScrollLinklabel="17"/><SupScrollLinklabel="20"/> . Here's how it works:</p>
<p>CFTR functions primarily as a chloride ion channel. It is responsible for transporting chloride ions (Cl⁻) across epithelial cell membranes<SupScrollLinklabel="20"/> . Here's how it works:</p>
<ol>
<li><b>Regulation by phosphorylation</b>: The R domain must first be phosphorylated by PKA to allow channel activation. This phosphorylation is often triggered by cyclic AMP (cAMP), a signaling molecule <SupScrollLinklabel="21"/> .</li>
<li><b>Opening the channel</b>: Once the R domain is phosphorylated, ATP binds to the NBDs, causing conformational changes that open the chloride channel <SupScrollLinklabel="18"/> .</li>
<li><b>Chloride transport</b>: With the channel open, chloride ions move from inside the cell to the outside. This movement of chloride helps draw water out of the cell, thinning mucus and maintaining proper hydration of the epithelial surfaces <SupScrollLinklabel="19"/><SupScrollLinklabel="20"/> .</li>
<li><b>Closing the channel</b>: Hydrolysis of ATP causes the channel to close after a certain period, tightly regulating chloride transport<SupScrollLinklabel="17"/>.</li>
<li><b>Closing the channel</b>: Hydrolysis of ATP causes the channel to close after a certain period, tightly regulating chloride transport.</li>
</ol>
<p>CFTR plays a critical role in maintaining the fluid balance on the surfaces of tissues such as the airways, digestive tract and sweat glands. By allowing chloride ions to flow out of the cells, CFTR ensures that water follows, preventing the accumulation of thick, sticky mucus <SupScrollLinklabel="19"/> .</p>
<H4text="CFTR in Cystic Fibrosis"id="CFTR-in-cftr"/>
