Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder that is characterized by the abnormal accumulation of surfactant within the alveoli. Alveolar macrophages (AMs) have been identified as playing a pivotal role in the pathogenesis of PAP. In most of PAP cases, the disease is triggered...
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| Format: | Article |
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Frontiers Media S.A.
2023-06-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1195988/full |
| _version_ | 1797804464652943360 |
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| author | Xinmei Huang Xinmei Huang Mengshu Cao Mengshu Cao Mengshu Cao Mengshu Cao Yonglong Xiao Yonglong Xiao |
| author_facet | Xinmei Huang Xinmei Huang Mengshu Cao Mengshu Cao Mengshu Cao Mengshu Cao Yonglong Xiao Yonglong Xiao |
| author_sort | Xinmei Huang |
| collection | DOAJ |
| description | Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder that is characterized by the abnormal accumulation of surfactant within the alveoli. Alveolar macrophages (AMs) have been identified as playing a pivotal role in the pathogenesis of PAP. In most of PAP cases, the disease is triggered by impaired cholesterol clearance in AMs that depend on granulocyte-macrophage colony-stimulating factor (GM-CSF), resulting in defective alveolar surfactant clearance and disruption of pulmonary homeostasis. Currently, novel pathogenesis-based therapies are being developed that target the GM-CSF signaling, cholesterol homeostasis, and immune modulation of AMs. In this review, we summarize the origin and functional role of AMs in PAP, as well as the latest therapeutic strategies aimed at addressing this disease. Our goal is to provide new perspectives and insights into the pathogenesis of PAP, and thereby identify promising new treatments for this disease. |
| first_indexed | 2024-03-13T05:37:34Z |
| format | Article |
| id | doaj.art-718c2971ea094072b8604dc788aa1d1f |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-03-13T05:37:34Z |
| publishDate | 2023-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-718c2971ea094072b8604dc788aa1d1f2023-06-14T05:38:47ZengFrontiers Media S.A.Frontiers in Immunology1664-32242023-06-011410.3389/fimmu.2023.11959881195988Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategiesXinmei Huang0Xinmei Huang1Mengshu Cao2Mengshu Cao3Mengshu Cao4Mengshu Cao5Yonglong Xiao6Yonglong Xiao7Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, ChinaNanjing Institute of Respiratory Diseases, Nanjing, ChinaDepartment of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, ChinaNanjing Institute of Respiratory Diseases, Nanjing, ChinaDepartment of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital Clinical College of Traditional Chinese and Western Medicine, Nanjing University of Chinese Medicine, Nanjing, ChinaDepartment of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, Nanjing, ChinaDepartment of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, ChinaNanjing Institute of Respiratory Diseases, Nanjing, ChinaPulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder that is characterized by the abnormal accumulation of surfactant within the alveoli. Alveolar macrophages (AMs) have been identified as playing a pivotal role in the pathogenesis of PAP. In most of PAP cases, the disease is triggered by impaired cholesterol clearance in AMs that depend on granulocyte-macrophage colony-stimulating factor (GM-CSF), resulting in defective alveolar surfactant clearance and disruption of pulmonary homeostasis. Currently, novel pathogenesis-based therapies are being developed that target the GM-CSF signaling, cholesterol homeostasis, and immune modulation of AMs. In this review, we summarize the origin and functional role of AMs in PAP, as well as the latest therapeutic strategies aimed at addressing this disease. Our goal is to provide new perspectives and insights into the pathogenesis of PAP, and thereby identify promising new treatments for this disease.https://www.frontiersin.org/articles/10.3389/fimmu.2023.1195988/fullalveolar macrophage (AM)pulmonary alveolar proteinosis (PAP)granulocyte-macrophage colony-stimulating factor (GM-CSF)pulmonary homeostasistherapeutic strategies |
| spellingShingle | Xinmei Huang Xinmei Huang Mengshu Cao Mengshu Cao Mengshu Cao Mengshu Cao Yonglong Xiao Yonglong Xiao Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies Frontiers in Immunology alveolar macrophage (AM) pulmonary alveolar proteinosis (PAP) granulocyte-macrophage colony-stimulating factor (GM-CSF) pulmonary homeostasis therapeutic strategies |
| title | Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies |
| title_full | Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies |
| title_fullStr | Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies |
| title_full_unstemmed | Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies |
| title_short | Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies |
| title_sort | alveolar macrophages in pulmonary alveolar proteinosis origin function and therapeutic strategies |
| topic | alveolar macrophage (AM) pulmonary alveolar proteinosis (PAP) granulocyte-macrophage colony-stimulating factor (GM-CSF) pulmonary homeostasis therapeutic strategies |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2023.1195988/full |
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