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

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.

Protocols & Documentation

  • PBMC Isolation from Whole Blood

    Standard operating procedure for isolating peripheral blood mononuclear cells from whole blood using density-gradient separation.

    Download
  • Immune Cell Isolation

    Guidelines for isolating immune cell populations with validated enrichment steps, purity checkpoints, and documentation.

    Download
  • Choosing the Right PBMC Configuration

    Reference guide comparing PBMC formats, cryopreservation states, and study-fit recommendations for your application.

    Download
  • Thawing Cryopreserved PBMC

    Validated thawing protocol to recover viable cryopreserved PBMCs while minimizing activation and loss of function.

    Download
  • Informed Consent Form (ICF)

    Available upon request — confirms donor consent for research use and downstream commercialization.

PBMC

Frequently Asked Questions

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Are samples IRB approved?

1 Answer

YES – All collections are conducted under IRB-approved protocols and electronic informed consent. Sanguine utilizes Advarra and WCG IRB for oversight.

YES – all our products are research use only (RUO).

Storage depends on sample type but often cryopreserved samples stored in liquid nitrogen can be stored for years.

Extensive customization is available. To discuss your project request a quote or email us at learnmore@sanguinebio.com.

Sanguine offers flexible prospective collection services tailored to fit your research. For more information and to request a quote, please see: our prospective biospecimen collection services page.

Timelines depend on condition, sample type, and I/E criteria but we often start collection within 2 weeks of signed agreement.

YES – we have in-stock inventory. You can also email us at learnmore@sanguinebio.com as we continually get new samples in our inventory.

Each sample from a different individual – Unique donor means each sample comes from a different person, ensuring biological diversity in your study.

YES – Unique donors can be specified and guaranteed based on your requirements.

For information about sample quality, please see: Quality and Compliance

YES – “IRB-approved collection protocols” and IRB approval documentation available upon request. Sanguine utilizes two internationally-recognized IRBs (Advarra and WCG IRB) for review and approval.

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Need a custom PBMC cohort?

Our scientific team can scope prospective collections with donor-specific I/E criteria, typically starting within 2 weeks of agreement.

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