Table 2 Differentially regulated genes and ontology: Group 1 pulmonary arterial hypertension vs. controls.

CFAP92

Cilia and flagella associated protein 92 (putative)

Aberrant expression associated with deficiency of Acyl-CoA Dehydrogenase (the first enzyme involved in mitochondrial fatty-acid oxidation), associated with mitochondrial Complex I deficiency

SNORA4

Small nucleolar RNA, H/ACA box 4

Involved in RNA processing, 17β-estradiol results in increased SNORA4 mRNA, snoRNAs are involved in cancer pathogenesis

TVP23A

Trans-golgi network vesicle protein 23 homolog A

Protein secretion, vesicle-mediated transport, vacuole, integral component of Golgi membrane, plasma membrane, enhanced protein expression in many types of cancers

SPIN3

Spindlin family member 3

Methylated histone binding, regulation of transcription

PNISR

PNN interacting serine and arginine rich protein

Participates in RNA binding, active in presynaptic active zone

PAPSS1

3′-Phosphoadenosine 5′-phosphosulfate synthase 1

Bifunctional enzyme: transfer of sulfate to ATP and transfer of phosphate from ATP to APS, required for biosynthesis of sulfated L-selectin ligands in endothelial cells

Hop pathway in cardiac development

 GATA4

Transcription factor in BMP pathway, transcriptional activator of ANF in cooperation with NKX2-5

 NKX2-5

Transcriptional activator of ANF in cooperation with GATA4

ALK in cardiac myocytes

 GATA4

Transcription factor in BMP pathway, transcriptional activator of ANF in cooperation with NKX2-5

 NKX2-5

Transcriptional activator of ANF in cooperation with GATA4

 ACVR1

BMPR1, transduces signals of BMPs, forms a complex with BMPs (including BMPR2) and then recruits SMAD, activates canonical SMAD signaling. phosphorylates SMAD1/5/8, suppresses TGFbeta/activin pathway,

Leukocyte transendothelial migration

 ACTN1

Actin filament bundle assembly, platelet degranulation, regulation of Bcl-2-apoptotic process

 CTNND1

Delta-catenin, negative regulation of Wnt signaling pathway (differentiation, proliferation, migration), cell–cell adhesion,

 RAC2

Augments production of ROS by NOX1 and 2, positive regulation of proliferation

 VASP

Substrate of BCR-ABL oncoprotein, actin-associated protein, cell migration, platelet activation, cytoskeleton remodeling, actin filament elongation

Fanconi anemia pathway

 SLX1A

DNA endonuclease

 SLX1B

DNA endonuclease

 CENPS

DNA binding component of the Fanconi Anemia core complex, leads to monoubiquitination of the complex in response to DNA damage, prevents chromosomal breakage