Jyotsana Patel², Poonam Bharti², Aryan Agrahari², Mukul Kumar Yadav², Shivendra Shukla², Ritik Dixit², Surisetti Divya¹* and Esha Yadav³

¹Assistant Professor, Department of Pharmacology, Axis Institute of Pharmacy, Rooma, Kanpur, Uttar Pradesh, India
²Student of B. Pharmacy, Department of Pharmacology, Axis Institute of Pharmacy, Rooma, Kanpur, Uttar Pradesh, India
³Professor, Department of Pharmacology, Axis Institute of Pharmacy, Rooma, Kanpur, Uttar Pradesh, India

*Corresponding Author: Surisetti Divya, Assistant Professor, Department of Pharmacology, Axis Institute of Pharmacy, Rooma, Kanpur, Uttar Pradesh, India.

Received: April 08, 2024; Published: April 24, 2024

Abstract

Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive pattern, arising from mutations in the gene responsible for encoding the cystic fibrosis transmembrane conductance regulator (CFTR). While CF primarily manifests with lung disease, it also affects other organs such as the pancreas, intestines, and skin, often utilized for early diagnostic testing. In CF, lung disease represents the primary cause of morbidity and mortality. Despite being largely infectious in nature, the associated inflammation is severe and ineffective in clearing pathogens. This persistent, high-intensity inflammation leads to structural damage in the airways and compromised lung function, ultimately culminating in respiratory failure and death. Autopsy cases of CF reveal multiorgan involvement, with some rarely observed changes. Defective inflammatory responses associated with CFTR deficiency include dysregulation of both innate and acquired immunity, abnormalities in cell membrane lipids, signaling defects in various transcription factors, and altered responses of kinases and toll-like receptors. Recent advancements in understanding the molecular mechanisms underlying CF have facilitated the development of CFTR modulator therapies, marking significant progress in CF treatment. These targeted therapies represent a shift towards precision medicine and are anticipated to further enhance survival rates in the foreseeable future.

Keywords: Cystic Fibrosis; CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Mutations; Lung Disease; Inflammation; CFTR Therapies; Precision

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Citation

Citation: Surisetti Divya., et al. “Deciphering the Intricacies of Cystic Fibrosis: An Advanced Exploration and Comprehensive Analysis". Acta ScientificActa Scientific Pharmaceutical Sciences 8.5 (2024): 14-26.

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Copyright: © 2024 Surisetti Divya., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.