Pulmonary Arterial Hypertension PBMC

About Pulmonary Arterial Hypertension PBMC

Pulmonary Arterial Hypertension PBMC for Pulmonary Vascular Remodeling Research

Pulmonary Arterial Hypertension PBMC are isolated from IRB-consented donors with clinically confirmed PAH and processed under standardized SOPs. Pulmonary arterial hypertension is WHO/ESC group 1 pulmonary hypertension: a disease of progressive pulmonary vascular remodeling, not an autoimmune disease. These cells support hereditary-PAH genotyping, immune and transcriptomic profiling of the inflammatory contribution to remodeling, and derivation of patient-specific vascular cell models.

PAH Biology and Why PBMC

• Vascular remodeling mechanism: endothelial dysfunction, pulmonary artery smooth muscle cell proliferation with resistance to apoptosis, adventitial thickening, and plexiform lesions progressively obliterate the distal pulmonary arteries, raising pulmonary vascular resistance and right ventricular afterload.
• BMPR2 and hereditary PAH: loss-of-function variants in BMPR2 are the major hereditary cause and are also found in a subset of idiopathic cases; other implicated genes include EIF2AK4, ACVRL1, ENG, TBX4, and SOX17. PBMCs are a convenient source of high-quality genomic DNA and RNA for this genotyping.
• Immune and inflammatory contribution: perivascular infiltration by macrophages, T cells, B cells, and dendritic cells, together with an altered circulating cytokine milieu, is a recognized contributor to remodeling — making circulating mononuclear cells a relevant and accessible readout.
• Connective tissue disease-associated PAH: an important subgroup within group 1, most notably systemic sclerosis-associated PAH, where autoimmunity contributes to the vascular disease. This is a subgroup, not the mechanism of PAH as a whole.
• iPSC derivation: PBMCs can be reprogrammed to induced pluripotent stem cells and differentiated into pulmonary artery endothelial and smooth muscle cells, enabling BMP/TGF-beta signaling, apoptosis, and angiogenesis assays in the donor’s own genotype.
• Transcriptomics: bulk and single-cell RNA sequencing of circulating immune populations for signature discovery and treatment-response research.

Donor Stratification Available

• PAH subtype: idiopathic, hereditary, connective tissue disease-associated (including systemic sclerosis), or congenital heart disease-associated
• BMPR2 or other hereditary PAH variant status where documented
• WHO functional class I-IV
• Treatment status: endothelin receptor antagonist, PDE5 inhibitor or soluble guanylate cyclase stimulator, prostacyclin analogue, as monotherapy or combination therapy
• Treatment-naive versus treated donors
• Right heart catheterization-confirmed diagnosis where documented
• Matched healthy controls available

Product Features

• Research Use Only (RUO) PBMCs
• PBMC isolated from clinically confirmed pulmonary arterial hypertension donors
• Rigorous donor screening and sample quality checks
• Standardized collection and processing SOPs
• IRB-approved protocols and informed donor consent
• Suitable for downstream applications such as flow cytometry, RNA-seq, ELISA, and qPCR
• Screen LeukoLot™ available prior to bulk orders

De-identified Donor Data

• Verified PAH diagnosis and diagnostic method
• Age, sex assigned at birth, race/ethnicity
• Medications, including PAH-specific therapy
• Functional class and disease severity/stage
• Optional: comorbidities, lab values, and clinical history

Applications

• BMPR2 and hereditary PAH gene panel sequencing and variant interpretation
• iPSC derivation and differentiation to pulmonary artery endothelial and smooth muscle cells
• Immunophenotyping of circulating monocyte, T cell, and B cell populations
• Bulk and single-cell transcriptomics for signature discovery
• Mechanistic studies of BMP/TGF-beta signaling in patient-derived cells
• Comparative studies across idiopathic, hereditary, and CTD-associated PAH
• Pharmacodynamic and treatment-response research in donors on approved PAH therapies
• Biomarker discovery and validation

Other Pulmonary Arterial Hypertension Specimen Types

• Pulmonary Arterial Hypertension Plasma
• Pulmonary Arterial Hypertension Serum
• Pulmonary Arterial Hypertension Whole Blood

Compliance and Quality Assurance

• IRB-approved protocols
• Informed donor consent
• HIPAA-compliant donor data protection
• Standardized collection and processing SOPs

Ordering & Customization

Samples are available in standard and custom cell counts to meet your study design. For pricing, current availability, international shipments, or country-specific documentation requirements, contact learnmore@sanguinebio.com to ensure compliance with your local regulations. To browse other conditions and live stock status, visit our human PBMC page.