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Cells
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Molecular Pathogenesis of PH: Group 1 and Beyond
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Molecular Pathogenesis of PH: Group 1 and Beyond

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: closed (30 November 2023) | Viewed by 5090

Special Issue Editors

Dr. James West

E-Mail Website
Guest Editor
Vanderbilt University Medical Center, Nashville, TN, USA
Interests: BMPR2; pulmonary hypertension; vascular biology; mouse models
Special Issues, Collections and Topics in MDPI journals
Dr. Jair Antonio Tenorio Castaño

E-Mail Website
Guest Editor
Hospital Universitario La Paz, Madrid, Spain
Interests: molecular genetics; NGS; genetic diagnostic
Special Issues, Collections and Topics in MDPI journals
Dr. Vinicio A. De Jesus Perez

E-Mail Website
Guest Editor
School of Medicine, Stanford University, Stanford, CA, USA
Interests: pulmonary hypertension; pulmonary fibrosis; vascular biology; pericytes; endothelial cells; smooth muscle cells; mitochondria; drug-induced lung injury, health disparities; medical education
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Pulmonary hypertension (PH) is a disease associated with abnormally elevated pulmonary pressures and right heart failure that can lead to premature death if untreated. The World Health Organization (WHO) groups PH into five broad categories, based on the clinical etiology of the disease. These groups share many common clinical features, including elevated pulmonary vascular pressures and remodeling of the pulmonary vasculature, but for most groups beyond Group 1, details of molecular contribution and even physiologic courses are still understudied.

This Special Issue represents the latest research efforts to update the community on mechanisms of disease in any of the five groups of pulmonary hypertension. An understanding of both the commonalities and differences between groups will assist in the search for treatment targets for disease of any etiology. 

Dr. James West
Dr. Jair Antonio Tenorio Castaño
Dr. Vinicio A. De Jesus Perez
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • group 1: pulmonary arterial hypertension
  • group 2: pulmonary hypertension due to left heart disease
  • group 3: pulmonary hypertension due to lung disease
  • group 4: chronic thromboembolic pulmonary hypertension (CTEPH)
  • group 5: pulmonary hypertension with unclear or multifactorial mechanisms

Published Papers (2 papers)

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Review

27 pages, 3233 KiB  
Review
The Role of Clonal Hematopoiesis of Indeterminant Potential and DNA (Cytosine-5)-Methyltransferase Dysregulation in Pulmonary Arterial Hypertension and Other Cardiovascular Diseases
by Isaac M. Emon, Ruaa Al-Qazazi, Michael J. Rauh and Stephen L. Archer
Cells 2023, 12(21), 2528; https://doi.org/10.3390/cells12212528 - 26 Oct 2023
Cited by 1 | Viewed by 1649
Abstract
DNA methylation is an epigenetic mechanism that regulates gene expression without altering gene sequences in health and disease. DNA methyltransferases (DNMTs) are enzymes responsible for DNA methylation, and their dysregulation is both a pathogenic mechanism of disease and a therapeutic target. DNMTs change [...] Read more.
DNA methylation is an epigenetic mechanism that regulates gene expression without altering gene sequences in health and disease. DNA methyltransferases (DNMTs) are enzymes responsible for DNA methylation, and their dysregulation is both a pathogenic mechanism of disease and a therapeutic target. DNMTs change gene expression by methylating CpG islands within exonic and intergenic DNA regions, which typically reduces gene transcription. Initially, mutations in the DNMT genes and pathologic DNMT protein expression were found to cause hematologic diseases, like myeloproliferative disease and acute myeloid leukemia, but recently they have been shown to promote cardiovascular diseases, including coronary artery disease and pulmonary hypertension. We reviewed the regulation and functions of DNMTs, with an emphasis on somatic mutations in DNMT3A, a common cause of clonal hematopoiesis of indeterminant potential (CHIP) that may also be involved in the development of pulmonary arterial hypertension (PAH). Accumulation of somatic mutations in DNMT3A and other CHIP genes in hematopoietic cells and cardiovascular tissues creates an inflammatory environment that promotes cardiopulmonary diseases, even in the absence of hematologic disease. This review summarized the current understanding of the roles of DNMTs in maintenance and de novo methylation that contribute to the pathogenesis of cardiovascular diseases, including PAH. Full article
(This article belongs to the Special Issue Molecular Pathogenesis of PH: Group 1 and Beyond)
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Graphical abstract

35 pages, 5074 KiB  
Review
The Role of Mitochondrial Dynamics and Mitotic Fission in Regulating the Cell Cycle in Cancer and Pulmonary Arterial Hypertension: Implications for Dynamin-Related Protein 1 and Mitofusin2 in Hyperproliferative Diseases
by Pierce Colpman, Asish Dasgupta and Stephen L. Archer
Cells 2023, 12(14), 1897; https://doi.org/10.3390/cells12141897 - 20 Jul 2023
Cited by 1 | Viewed by 2978
Abstract
Mitochondria, which generate ATP through aerobic respiration, also have important noncanonical functions. Mitochondria are dynamic organelles, that engage in fission (division), fusion (joining) and translocation. They also regulate intracellular calcium homeostasis, serve as oxygen-sensors, regulate inflammation, participate in cellular and organellar quality control [...] Read more.
Mitochondria, which generate ATP through aerobic respiration, also have important noncanonical functions. Mitochondria are dynamic organelles, that engage in fission (division), fusion (joining) and translocation. They also regulate intracellular calcium homeostasis, serve as oxygen-sensors, regulate inflammation, participate in cellular and organellar quality control and regulate the cell cycle. Mitochondrial fission is mediated by the large GTPase, dynamin-related protein 1 (Drp1) which, when activated, translocates to the outer mitochondrial membrane (OMM) where it interacts with binding proteins (Fis1, MFF, MiD49 and MiD51). At a site demarcated by the endoplasmic reticulum, fission proteins create a macromolecular ring that divides the organelle. The functional consequence of fission is contextual. Physiological fission in healthy, nonproliferating cells mediates organellar quality control, eliminating dysfunctional portions of the mitochondria via mitophagy. Pathological fission in somatic cells generates reactive oxygen species and triggers cell death. In dividing cells, Drp1-mediated mitotic fission is critical to cell cycle progression, ensuring that daughter cells receive equitable distribution of mitochondria. Mitochondrial fusion is regulated by the large GTPases mitofusin-1 (Mfn1) and mitofusin-2 (Mfn2), which fuse the OMM, and optic atrophy 1 (OPA-1), which fuses the inner mitochondrial membrane. Mitochondrial fusion mediates complementation, an important mitochondrial quality control mechanism. Fusion also favors oxidative metabolism, intracellular calcium homeostasis and inhibits cell proliferation. Mitochondrial lipids, cardiolipin and phosphatidic acid, also regulate fission and fusion, respectively. Here we review the role of mitochondrial dynamics in health and disease and discuss emerging concepts in the field, such as the role of central versus peripheral fission and the potential role of dynamin 2 (DNM2) as a fission mediator. In hyperproliferative diseases, such as pulmonary arterial hypertension and cancer, Drp1 and its binding partners are upregulated and activated, positing mitochondrial fission as an emerging therapeutic target. Full article
(This article belongs to the Special Issue Molecular Pathogenesis of PH: Group 1 and Beyond)
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