Mitochondrial Dysfunction in Pulmonary Fibrosis

Rangarajan, Sunad; Bernard, Karen; Thannickal, Victor J.

doi:10.1513/annalsats.201705-370aw

Cited by 73 publications

(61 citation statements)

References 107 publications

(107 reference statements)

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“…SIRT3 expression is diminished in LPS-induced ALI. SIRT3 is an NAD + -dependent deacetylase that is primarily localized to the mitochondria (25,26), and its deficiency has been linked to modulation of mitochondrial function and redox homeostasis (27,28). The role of SIRT3 in ALI is unclear.…”

Section: Resultsmentioning

confidence: 99%

SIRT3 diminishes inflammation and mitigates endotoxin-induced acute lung injury

Kurundkar¹,

Kurundkar²,

Bone³

et al. 2019

JCI Insight

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119

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Section: Resultsmentioning

confidence: 99%

SIRT3 diminishes inflammation and mitigates endotoxin-induced acute lung injury

Kurundkar¹,

Kurundkar²,

Bone³

et al. 2019

JCI Insight

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119

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“…AT2 cells have the highest number of mitochondria in the lungs due to their high metabolic demands, especially during lung injury and repair [72]. Disturbance and interference of AT2 mitochondrial biogenesis, functions, and homeostasis is a known profibrotic signal [73][74][75]. The imbalance of mitochondrial dynamic due to impaired mitophagy and accumulation of mtDNA damage or irregularities of protein homeostasis leads to ER stress and program cell death of AT2 cells [74,76].…”

Section: Mitochondrial Dysfunction Causes At2 Deathmentioning

confidence: 99%

Alveolar Epithelial Type II Cells as Drivers of Lung Fibrosis in Idiopathic Pulmonary Fibrosis

Parimon

Yao

Stripp

et al. 2020

IJMS

244

172

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: Alveolar epithelial type II cells (AT2) are a heterogeneous population that have critical secretory and regenerative roles in the alveolus to maintain lung homeostasis. However, impairment to their normal functional capacity and development of a pro-fibrotic phenotype has been demonstrated to contribute to the development of idiopathic pulmonary fibrosis (IPF). A number of factors contribute to AT2 death and dysfunction. As a mucosal surface, AT2 cells are exposed to environmental stresses that can have lasting effects that contribute to fibrogenesis. Genetical risks have also been identified that can cause AT2 impairment and the development of lung fibrosis. Furthermore, aging is a final factor that adds to the pathogenic changes in AT2 cells. Here, we will discuss the homeostatic role of AT2 cells and the studies that have recently defined the heterogeneity of this population of cells. Furthermore, we will review the mechanisms of AT2 death and dysfunction in the context of lung fibrosis.

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“…Mitochondrial-generated increase of ROS has been found to induce lung fibrosis [154,155]. Although a potentially vicious cycle of TGF-β and ROS interaction exists, where ROS activate TGF-β and TGF-β activates ROS [156][157][158]), TGF-β1 appears to be the most important trigger of mitochondrial (mtROS) production associated with the profibrotic phenotype reprogramming of lung cells [159]. This proposition is further supported by the observation that the deletion of NOX4 abrogates TGF-β1-induced fibrosis in mice [160].…”

Section: Mitochondrial Dysfunctionmentioning

confidence: 99%

Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis

Phan

Paliogiannis

Nasrallah

et al. 2020

Cell. Mol. Life Sci.

220

152

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Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, is a progressive, irreversible, and typically lethal disease characterized by an abnormal fibrotic response involving vast areas of the lungs. Given the poor knowledge of the mechanisms underpinning IPF onset and progression, a better understanding of the cellular processes and molecular pathways involved is essential for the development of effective therapies, currently lacking. Besides a number of established IPF-associated risk factors, such as cigarette smoking, environmental factors, comorbidities, and viral infections, several other processes have been linked with this devastating disease. Apoptosis, senescence, epithelial-mesenchymal transition, endothelial-mesenchymal transition, and epithelial cell migration have been shown to play a key role in IPF-associated tissue remodeling. Moreover, molecules, such as chemokines, cytokines, growth factors, adenosine, glycosaminoglycans, non-coding RNAs, and cellular processes including oxidative stress, mitochondrial dysfunction, endoplasmic reticulum stress, hypoxia, and alternative polyadenylation have been linked with IPF development. Importantly, strategies targeting these processes have been investigated to modulate abnormal cellular phenotypes and maintain tissue homeostasis in the lung. This review provides an update regarding the emerging cellular and molecular mechanisms involved in the onset and progression of IPF.

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Mitochondrial Dysfunction in Pulmonary Fibrosis

Cited by 73 publications

References 107 publications

SIRT3 diminishes inflammation and mitigates endotoxin-induced acute lung injury

SIRT3 diminishes inflammation and mitigates endotoxin-induced acute lung injury

Alveolar Epithelial Type II Cells as Drivers of Lung Fibrosis in Idiopathic Pulmonary Fibrosis

Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis

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