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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

BMPR2

PDCD4/caspase-3

Proliferating cell nuclear antigen

cyclin D1

Bcl-xL

DDAH1

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

SHIP-1

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

[32]

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

SMAD7 SMAD3 COL1A1

SSc

[33]

 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

NFATc1

CAMTA1

PTBP1

STAT3/NFAT signaling

PASMC proliferation, prodifferentiation, and survival

Inflammation

Pulmonary vascular remodeling

[36]

 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

Mice

Multiple

PH

[37]

 miR-181a-5p

Human pulmonary artery endothelial cells

PAH mice

Multiple

Vascular remodeling

[38]

 miR-29

 miR-29b

Mice

Mice pulmonary fibroblasts

TGF-β

CTGF

SMAD3

Pulmonary fibrosis

[39]

 miR-455-3p-1

Normal and PAH patients

FGF7

Inhibit the proliferation of PASMCs

[40]

 miR-223-3p

PASMCs

Rat

ITGB3

Pulmonary vascular remodeling

[41]

 miR-17/92 family

HEK293

Rodent

model of PH

BMPR2

STAT3

Development of PH

[42]

miRNA related to BMPR2 signaling pathway

 miR-204

PASMCs in both human and rodent PAH

SHP2

NFAT

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

LRP8

Pulmonary vascular stiffening

Extracellular matrix remodeling

[46]

 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