Amyotrophic Lateral Sclerosis PBMC

Amyotrophic Lateral Sclerosis PBMC for Neurodegeneration Research

Amyotrophic Lateral Sclerosis (ALS) PBMC products are sourced from IRB-consented donors clinically diagnosed with ALS and processed within 24 hours of collection under standardised conditions. ALS is a progressive neurodegenerative disease of the upper and lower motor neurons, characterised pathologically by cytoplasmic TDP-43 proteinopathy in the great majority of cases. These cryopreserved peripheral blood mononuclear cells support neurodegeneration research, peripheral immune characterisation and patient-derived cell model generation.

Motor Neuron Degeneration and What Peripheral Cells Contribute

• ALS is a neurodegenerative disorder, not an autoimmune one. The core pathology is motor neuron loss with TDP-43 mislocalisation and aggregation, accompanied by glial activation in the central nervous system.
• A subset of cases is familial, most commonly linked to the C9orf72 hexanucleotide repeat expansion and to variants in SOD1, TARDBP and FUS; PBMCs carry the donor genotype and so support genetic and functional follow-up.
• Peripheral immune cells in ALS show altered activation and regulatory T cell profiles reported across multiple cohorts, positioning PBMCs as a tractable window on the neuroinflammatory component of disease.
• PBMCs are a nucleated cell source, so they are appropriate for immunophenotyping by flow cytometry, single-cell and bulk transcriptomics, and functional stimulation assays.
• PBMCs are widely used as a starting material for induced pluripotent stem cell reprogramming, enabling patient-derived motor neuron and glial models carrying the donor’s genetic background.
• Monocyte and lymphocyte gene expression signatures are actively investigated as accessible peripheral correlates of disease progression, complementing CSF and neurofilament measures.

Donor Stratification Available

• Sporadic versus familial ALS where documented
• Known genotype where available, including C9orf72, SOD1, TARDBP and FUS
• Site of onset: bulbar versus limb onset
• Disease duration since symptom onset or diagnosis
• Functional status and treatment history, including riluzole and edaravone where reported
• Matched healthy controls available

Product Features

• Research Use Only (RUO), cryopreserved PBMCs
• Clinically confirmed ALS donors
• Processed within 24 hours of collection
• Stored using CryoStor® CS10 freezing media
• Custom aliquot sizes available on request
• IRB-approved protocols and electronic informed consent

De-identified Donor Data

• Verified ALS diagnosis
• Donor demographics: age, sex assigned at birth, race/ethnicity
• Self-reported allergies and infectious disease history
• Medication and treatment history where reported
• Additional ALS-specific data available upon request

Applications

• Immunophenotyping of lymphocyte, monocyte and regulatory T cell populations by flow cytometry
• Bulk and single-cell RNA sequencing of peripheral immune populations
• iPSC reprogramming and patient-derived motor neuron model generation
• Genotyping and functional characterisation of ALS-associated variants
• Neuroinflammation and cytokine release assay development
• Peripheral biomarker discovery for disease progression
• Drug screening and target engagement studies in patient-derived cells
• Proteomic profiling of patient-derived mononuclear cells

Other Amyotrophic Lateral Sclerosis Specimen Types

• Amyotrophic Lateral Sclerosis Plasma
• Amyotrophic Lateral Sclerosis Serum
• Amyotrophic Lateral Sclerosis Whole Blood

Compliance and Quality Assurance

• IRB-approved collections and protocols
• 21 CFR Part 11 – compliant e-consent system
• HIPAA-compliant donor data protection

Ordering & Customization

Amyotrophic Lateral Sclerosis PBMC samples are shipped on dry ice and available in custom aliquots. For pricing, current availability, international orders or regulatory requirements, please contact learnmore@sanguinebio.com to confirm documentation and compliance needs. To browse donor cohorts across our full inventory, visit our human PBMCs page.