Cystic Fibrosis antibodies

and ELISA kits, proteins related to Cystic Fibrosis.

Introduction to Cystic Fibrosis

Cystic Fibrosis (CF) is a serious genetic disorder that primarily affects the lungs and digestive system. Caused by mutations in the CFTR gene, CF leads to the production of thick, sticky mucus that clogs airways and traps harmful bacteria, resulting in chronic respiratory infections and inflammation. This persistent lung damage can severely impair breathing and reduce quality of life. Additionally, the thick mucus obstructs the pancreas, hindering the release of essential enzymes needed for digestion and nutrient absorption. Ongoing research into CF is exploring innovative treatments, including the development of targeted antibodies, to address the underlying molecular causes of the disease. These advancements aim to improve patient outcomes, enhance lung function, and extend the lifespan of those living with Cystic Fibrosis, offering renewed hope for effective management and eventual cures.

Cystic Fibrosis biomarkers

PB10058

Anti-Neutrophil Elastase/ELANE Antibody Picoband®, Neutrophil infiltration and NETs release within AAA lesions. A IF staining for neutrophil marker (Ly6G) was performed on ...

A01413-1

Anti-epithelial Sodium Channel alpha/SCNN1A Antibody Picoband®, IF analysis of SCNN1A using anti-SCNN1A antibody (A01413-1).
SCNN1A was detected in immunocytochemical s...

PA2303

Anti-Mucin 5AC/MUC5AC Antibody Picoband®, IHC analysis of Mucin 5AC using anti-Mucin 5AC antibody (PA2303).
Mucin 5AC was detected in paraffin-embedded section of human gastric can...

Protein Name	Gene Name	Function
Cystic Fibrosis Transmembrane Conductance Regulator	CFTR	Chloride channel for ion transport in epithelial cells
Epithelial Sodium Channel α	SCNN1A	Regulates sodium absorption and fluid balance
Epithelial Sodium Channel β	SCNN1B	Functions with ENaCα in sodium transport
Epithelial Sodium Channel γ	SCNN1G	Works with ENaCα and ENaCβ in sodium regulation
Mucin 5AC	MUC5AC	Mucin protein involved in mucus production
Mucin 5B	MUC5B	Mucin protein essential for mucus structure
Interleukin-6	IL6	Pro-inflammatory cytokine involved in immune responses
Neutrophil Elastase	ELANE	Protease from neutrophils causing tissue damage
Tumor Necrosis Factor-alpha	TNF	Pro-inflammatory cytokine mediating inflammation
Transforming Growth Factor-beta	TGFB1	Involved in tissue fibrosis and remodeling
SLC26A9	SLC26A9	Chloride channel implicated in ion transport
Interleukin-1beta	IL1B	Pro-inflammatory cytokine involved in immune response
Interleukin-17	IL17A	Pro-inflammatory cytokine involved in neutrophil recruitment
Macrophage Inflammatory Protein-1alpha	CCL3	Chemokine involved in recruiting immune cells
Kallikrein-related Peptidase 5	KLK5	Protease involved in skin barrier function and inflammation
Vascular Endothelial Growth Factor A	VEGFA	Regulates vascular permeability and angiogenesis
Galectin-3	LGALS3	Involved in cell adhesion and immune response
Interleukin-13	IL13	Pro-inflammatory cytokine involved in airway inflammation

Important Mechanisms

CFTR Protein Function and Modulation

Cystic Fibrosis (CF) is primarily caused by mutations in the CFTR gene, which encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This protein functions as a chloride channel, regulating the movement of ions and water across epithelial cell membranes in organs such as the lungs, pancreas, and intestines. Understanding the structure and function of CFTR is crucial for elucidating how specific mutations disrupt its normal activity, leading to the thick mucus characteristic of CF. Research in this subarea focuses on identifying the molecular mechanisms by which different CFTR mutations impair protein folding, trafficking, and channel function. Additionally, scientists are developing and optimizing therapeutic agents known as modulators—such as correctors and potentiators—that can enhance the proper folding and activity of the defective CFTR protein. These advancements not only improve our fundamental knowledge of CF pathophysiology but also pave the way for personalized medicine approaches, allowing treatments to be tailored to the specific genetic variants present in individual patients.

Inflammatory Pathways and Lung Disease

Chronic lung inflammation is a central feature of Cystic Fibrosis, leading to progressive respiratory decline and reduced quality of life. This subarea of research delves into the inflammatory processes that exacerbate lung damage in CF patients. Studies focus on the role of immune cells, such as neutrophils and macrophages, and the release of pro-inflammatory cytokines and chemokines that perpetuate a cycle of inflammation and infection. Understanding these pathways is essential for identifying potential therapeutic targets to mitigate excessive inflammation without compromising the necessary immune response to pathogens like Pseudomonas aeruginosa. Researchers are also investigating the interplay between microbial colonization and the host immune system, aiming to develop strategies that can effectively manage chronic infections while controlling inflammatory responses. By targeting the underlying mechanisms of inflammation, this research seeks to preserve lung function, slow disease progression, and enhance the overall respiratory health of individuals living with Cystic Fibrosis.