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Table 1 A List of microRNAs in this study

From: The role of TGF-β or BMPR2 signaling pathway-related miRNA in pulmonary arterial hypertension and systemic sclerosis

miRNA Experimental system Targets Function References
Putative miRNAs related to PAH and SSc
 miR-21 Human PAECs and PASMCs KLF4, Smad-8 PASMC differentiation and inhibition of PASMC proliferation [22]
 miR-21 Human PAECs
Rodent model of PH
Proliferating cell nuclear antigen
cyclin D1
PAH [23,24,25,26]
 miR-21 Fibroblasts SMAD7 Skin fibrosis [27]
 let-7g Human patients with SSc-PH multiple PAH [28]
 let-7a Human or mouse skin fibroblasts Type I collagen Tissue fibrosis in the skin [29]
 miR-155 Skin of mice CK1α
Dermal fibrosis [30]
 miR-29a Skin biopsy and fibroblast samples from SSc patients and healthy controls
The mouse model of bleomycin-induced skin fibrosis
Type I and type III collagen Fibrogenesis of SSc [31]
 miR-140-5p Marrow stromal cells TGF-βR1 TGF-βR1 regulation
Adipocyte differentiation
miRNA related to TGF-β-signaling pathway
 miR-155 Lung fibroblasts and PBMC from SSc-ILD patients Multiple Dysregulated lung gene expression [30]
 miR-145 Normal and cutaneous scleroderma skin tissues
Human fibroblasts
 miR-145 PAH patients
Human PASMCs
Hypoxia-induced PAH rats
ABCA1 PASMCs proliferation and migration [32]
 miR-221-3p Human PASMC
PAH animal models
AXIN2 PASMC proliferation [34]
 miR-124 Pulmonary vascular and circulating progenitor endothelial cells isolated from patients PTBP1 Metabolic and proliferative abnormalities in PAH ECs [35]
 miR-124 Human PASMCs
Nonfamilial human PAH
PAH mouse and rat models
STAT3/NFAT signaling
PASMC proliferation, prodifferentiation, and survival
Pulmonary vascular remodeling
 miR-143 PAH patients
Human PASMCs
Hypoxia-induced PAH rats
ABCA1 PASMC proliferation and migration [32]
 miR-143-3p Calf models of PAH
PAH patients
Multiple PH [37]
 miR-181a-5p Human pulmonary artery endothelial cells
PAH mice
Multiple Vascular remodeling [38]
Mice pulmonary fibroblasts
Pulmonary fibrosis [39]
 miR-455-3p-1 Normal and PAH patients FGF7 Inhibit the proliferation of PASMCs [40]
 miR-223-3p PASMCs
ITGB3 Pulmonary vascular remodeling [41]
 miR-17/92 family HEK293
model of PH
Development of PH [42]
miRNA related to BMPR2 signaling pathway
 miR-204 PASMCs in both human and rodent PAH SHP2
PAH-PASMC proliferation and resistance to apoptosis [43]
 miR-130/301 family Pulmonary vessels and plasma from mammalian models and PH patients
PAECs and PASMCs in mice model
PPARγ Cell proliferation in PH [44]
 miR-130/301 family Human PASMCs and PAECs
Mice blood and lung tissue
PPARγ Cell proliferation in PH [45]
 miR-130/301 family Human PAECs PPARγ
Pulmonary vascular stiffening
Extracellular matrix remodeling
 miR-424 (322) The blood of PAH patients
Hypoxia-induced PAECs
Monocrotaline rat model of PH
SMURF1 Afterload of the right ventricle [47]
 miR-17/92 family Human PASMCs PDLIM5 PASMC proliferation and differentiation [48]
 miR-20a Mice
Human PASMCs
BMPR2 Prevent pulmonary arterial vascular remodeling [49]
  1. PAH pulmonary arterial hypertension, SSC systemic sclerosis, PAEC pulmonary artery endothelial cell, PASMC pulmonary artery smooth muscle cell, KLF4 Kruppel Like Factor 4, Smad-8 Mothers against decapentaplegic homolog 8, PH pulmonary hypertension, BMPR2 Bone Morphogenetic Protein Receptor Type II, PDCD4 Programmed Cell Death 4, Bcl-xL B cell lymphoma-extra large, DDAH1 dimethylarginine dimethylaminohydrolases 1, SMAD7 Mothers against decapentaplegic homolog 7, CK1α casein kinase 1α, SHIP-1 Src homology 2-containing inositol phosphatase-1, TGF-βR1 transforming growth factor-beta receptor type 1, SSc-ILD systemic sclerosis associated interstitial lung disease, SMAD7 Mothers against decapentaplegic homolog 7, SMAD3 Mothers against decapentaplegic homolog 3, COL1A1 Collagen Type I Alpha 1 Chain, ABCA1 ATP-binding cassette transporter A1, AXIN2 Axis inhibition protein 2, PTBP1 polypyrimidine tract-binding protein 1, NFATc1 nuclear factor of activated T cells 1, CAMTA1 calmodulin-binding transcription activator 1, STAT3 signal transducer and activator of transcription 3, NFAT nuclear factor of activated T cells, TGF-β Transforming growth factor-beta, CTGF connective tissue growth factor, FGF7 Fibroblast Growth Factor 7, ITGB3 Integrin alpha-V/beta-3, SHP2 Src homology region 2 domain-containing phosphatase-2, PPARγ Peroxisome proliferator- activated receptor gamma, LRP8 lipoprotein receptor-related protein 8, SMURF1 SMAD-specific E3 ubiquitin protein ligase 1, PDLIM5 PDZ And LIM Domain 5