Pulmonary Vasculopathy Associated with FIGF Gene Mutation

Bailey, Evan; Cui, Ye; Casey, Alicia; Stoler, Joan M.; Ai, Xingbin; Ma, Dongdong; Handin, Robert I.; Sliz, Piotr; Vargas, Sara O.; El–Chemaly, Souheil

doi:10.1016/j.ajpath.2016.09.008

Cited by 7 publications

(9 citation statements)

References 25 publications

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“…Recent findings have implicated VEGF-D in the pathogenesis of other pulmonary diseases. For example, a form of pulmonary vasculopathy, involving dilated or distended pulmonary arteries and veins, has been associated with a mutation of the gene encoding VEGF-D in humans [ 53 ]. The mutation resulted in increased dimerization of VEGF-D (VEGF-D dimers are considered more bioactive than VEGF-D monomers [ 10 ]), elevated VEGFR-2 signaling and aberrant angiogenesis.…”

Section: Disease Involvement: Humans and Model Systemsmentioning

confidence: 99%

Emerging Roles for VEGF-D in Human Disease

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Achen

2018

Biomolecules

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Blood vessels and lymphatic vessels are located in many tissues and organs throughout the body, and play important roles in a wide variety of prevalent diseases in humans. Vascular endothelial growth factor-D (VEGF-D) is a secreted protein that can promote the remodeling of blood vessels and lymphatics in development and disease. Recent fundamental and translational studies have provided insight into the molecular mechanisms by which VEGF-D exerts its effects in human disease. Hence this protein is now of interest as a therapeutic and/or diagnostic target, or as a potential therapeutic agent, in a diversity of indications in cardiovascular medicine, cancer and the devastating pulmonary condition lymphangioleiomyomatosis. This has led to clinical trial programs to assess the effect of targeting VEGF-D signaling pathways, or delivering VEGF-D, in angina, cancer and ocular indications. This review summarizes our understanding of VEGF-D signaling in human disease, which is largely based on animal disease models and clinicopathological studies, and provides information about the outcomes of recent clinical trials testing agonists or antagonists of VEGF-D signaling.

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Section: Disease Involvement: Humans and Model Systemsmentioning

confidence: 99%

Emerging Roles for VEGF-D in Human Disease

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Achen

2018

Biomolecules

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“…More recently, studies have shown that VEGF-D may also play critical roles in lung vascular development. For instance, VEGF-D 2/2 mice are protected from the effects of hyperoxia (8), and the mutation in human VEGF-D (V118M) leads to aberrant protein dimerization, resulting in increased angiogenesis and worsening oxygen diffusion (9).…”

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confidence: 99%

Lymphatic Changes in Respiratory Diseases: More than Just Remodeling of the Lung?

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Cui

Kidambi

et al. 2017

Am J Respir Cell Mol Biol

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Advances in our ability to identify lymphatic endothelial cells and differentiate them from blood endothelial cells have led to important progress in the study of lymphatic biology. Over the past decade, preclinical and clinical studies have shown that there are changes to the lymphatic vasculature in nearly all lung diseases. Efforts to understand the contribution of lymphatics and their growth factors to disease initiation, progression, and resolution have led to seminal findings establishing critical roles for lymphatics in lung biology spanning from the first breath after birth to asthma, tuberculosis, and lung transplantation. However, in other diseases, it remains unclear if lymphatics are part of the overall lung remodeling process or real contributors to disease pathogenesis. The goal of this Translational Review is to highlight some of the advances in our understanding of the role(s) of lymphatics in lung disease and shed light on the critical needs and unanswered questions that might lead to novel translational applications.

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“…Our findings corroborate studies showing lymphangiogenesis in a HDM model of respiratory allergies in rats 6 , and studies by Kretschmer et al demonstrating that house dust mite challenge leads to lymphangiogenesis and T cell infiltration in murine lungs 37 . Other studies have also demonstrated that lymphangiogenesis occurs more broadly in chronic airway inflammation, particularly during diseases such as lymphangioleiomyomatosis, and in certain regions within lungs of idiopathic pulmonary fibrosis patients 7 , 22 , 23 , 37 – 40 . Interestingly, fatal asthma in humans, where fibrosis has occurred in much of the lung, is associated with fewer lymphatic vessels in the lungs 9 , 25 , 35 .…”

Section: Discussionmentioning

confidence: 96%