Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches
Idiopathic pulmonary fibrosis (IPF) is a life-threatening and progressive interstitial lung disease. Healthy pulmonary tissues were replaced by an altered extracellular matrix (ECM) and destroyed the alveolar architecture, which led to the disruption of gas exchange, decreased lung compliance, and u...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2023-12-01
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| Series: | Medicine in Drug Discovery |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2590098623000179 |
| _version_ | 1797449430874456064 |
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| author | Ilma Shakeel Mohammad Afzal Asimul Islam Sukhwinder Singh Sohal Md. Imtaiyaz Hassan |
| author_facet | Ilma Shakeel Mohammad Afzal Asimul Islam Sukhwinder Singh Sohal Md. Imtaiyaz Hassan |
| author_sort | Ilma Shakeel |
| collection | DOAJ |
| description | Idiopathic pulmonary fibrosis (IPF) is a life-threatening and progressive interstitial lung disease. Healthy pulmonary tissues were replaced by an altered extracellular matrix (ECM) and destroyed the alveolar architecture, which led to the disruption of gas exchange, decreased lung compliance, and ultimately led to the failure of respiration and death. IPF is associated with dyspnoea and cough, eventually impairing the quality of a large population worldwide, with the incidence rate increasing drastically with age. The IPF involves a complex pathophysiology; thus, it is necessary to understand the precise molecular mechanism for lung fibrosis to develop novel therapeutic options. Different kinases, including p38 mitogen-activated protein kinase, JNK (c-Jun N-terminal kinase), and SAPKs (Stress-activated protein kinases), were ubiquitously expressed in numerous variety of cells and were activated in response to inflammatory stimuli, cellular environmental stress, and stimuli for apoptosis. After many failed drugs for IPF in clinical trials, two drugs, namely nintedanib and pirfenidone, were approved, which helped to slow the disease progression. However, the prognosis of IPF remains poor, which leads to continuing the search for better drugs, targeting to either reduce or halt the disease progression. The current review summarises the pathophysiology, signaling, and role of different kinases, therapeutic interventions, investigational therapies, and biomarkers for IPF. A better understanding of the pathophysiology of IPF will be helpful to understand disease mechanisms, discovering potential biomarkers, and development of effective therapeutics to handle patients. |
| first_indexed | 2024-03-09T14:25:01Z |
| format | Article |
| id | doaj.art-8d3478839a7d40939021c76628ef2142 |
| institution | Directory Open Access Journal |
| issn | 2590-0986 |
| language | English |
| last_indexed | 2024-03-09T14:25:01Z |
| publishDate | 2023-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Medicine in Drug Discovery |
| spelling | doaj.art-8d3478839a7d40939021c76628ef21422023-11-28T07:26:57ZengElsevierMedicine in Drug Discovery2590-09862023-12-0120100167Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approachesIlma Shakeel0Mohammad Afzal1Asimul Islam2Sukhwinder Singh Sohal3Md. Imtaiyaz Hassan4Centre for Interdisciplinary Research in Basic Sciences, Jamia Millia Islamia, New Delhi, India; Department of Zoology, Aligarh Muslim University, Aligarh, IndiaDepartment of Zoology, Aligarh Muslim University, Aligarh, IndiaCentre for Interdisciplinary Research in Basic Sciences, Jamia Millia Islamia, New Delhi, IndiaRespiratory Translational Research Group, Department of Laboratory Medicine, School of Health Sciences, College of Health and Medicine, University of Tasmania, Launceston, Tasmania 7248, AustraliaCentre for Interdisciplinary Research in Basic Sciences, Jamia Millia Islamia, New Delhi, India; Corresponding author at: Centre for Interdisciplinary Research in Basic Sciences, Jamia Millia Islamia, Jamia Nagar, New Delhi 110025, India.Idiopathic pulmonary fibrosis (IPF) is a life-threatening and progressive interstitial lung disease. Healthy pulmonary tissues were replaced by an altered extracellular matrix (ECM) and destroyed the alveolar architecture, which led to the disruption of gas exchange, decreased lung compliance, and ultimately led to the failure of respiration and death. IPF is associated with dyspnoea and cough, eventually impairing the quality of a large population worldwide, with the incidence rate increasing drastically with age. The IPF involves a complex pathophysiology; thus, it is necessary to understand the precise molecular mechanism for lung fibrosis to develop novel therapeutic options. Different kinases, including p38 mitogen-activated protein kinase, JNK (c-Jun N-terminal kinase), and SAPKs (Stress-activated protein kinases), were ubiquitously expressed in numerous variety of cells and were activated in response to inflammatory stimuli, cellular environmental stress, and stimuli for apoptosis. After many failed drugs for IPF in clinical trials, two drugs, namely nintedanib and pirfenidone, were approved, which helped to slow the disease progression. However, the prognosis of IPF remains poor, which leads to continuing the search for better drugs, targeting to either reduce or halt the disease progression. The current review summarises the pathophysiology, signaling, and role of different kinases, therapeutic interventions, investigational therapies, and biomarkers for IPF. A better understanding of the pathophysiology of IPF will be helpful to understand disease mechanisms, discovering potential biomarkers, and development of effective therapeutics to handle patients.http://www.sciencedirect.com/science/article/pii/S2590098623000179Idiopathic pulmonary fibrosisCellular signalingBiomarkersKinasesDrug target |
| spellingShingle | Ilma Shakeel Mohammad Afzal Asimul Islam Sukhwinder Singh Sohal Md. Imtaiyaz Hassan Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches Medicine in Drug Discovery Idiopathic pulmonary fibrosis Cellular signaling Biomarkers Kinases Drug target |
| title | Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches |
| title_full | Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches |
| title_fullStr | Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches |
| title_full_unstemmed | Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches |
| title_short | Idiopathic pulmonary fibrosis: Pathophysiology, cellular signaling, diagnostic and therapeutic approaches |
| title_sort | idiopathic pulmonary fibrosis pathophysiology cellular signaling diagnostic and therapeutic approaches |
| topic | Idiopathic pulmonary fibrosis Cellular signaling Biomarkers Kinases Drug target |
| url | http://www.sciencedirect.com/science/article/pii/S2590098623000179 |
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