Cryopyrin-Associated Periodic Syndrome Serum

About Cryopyrin-Associated Periodic Syndrome Serum

Cryopyrin-Associated Periodic Syndrome Serum for Autoinflammatory Disease Research

Our CAPS Serum is sourced from IRB-consented donors clinically diagnosed with cryopyrin-associated periodic syndrome (CAPS) and processed and frozen within 24 hours of collection to preserve cytokines, acute-phase proteins, and metabolites. CAPS is a monogenic autoinflammatory disease of innate immunity – it is not autoimmune, and there are no disease-defining autoantibodies – and serum is the appropriate matrix for its IL-1-driven soluble mediators.

CAPS Biology and Why Serum

• CAPS is caused by gain-of-function variants in NLRP3, which encodes the innate immune sensor cryopyrin. The mutant protein assembles the NLRP3 inflammasome constitutively or at an inappropriately low activation threshold.
• Inflammasome assembly activates caspase-1, which cleaves pro-IL-1beta and pro-IL-18 into their mature, secreted forms and triggers gasdermin D-mediated inflammatory cell death. IL-1beta is the credible, mechanistically central serum-side analyte.
• Because the defect lies in innate sensing, CAPS involves no autoantigen, no autoreactive lymphocyte clone, and no autoantibody production. Adaptive-immunity framing does not apply to this disease.
• The clinical spectrum spans three overlapping phenotypes of increasing severity: familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID/CINCA), with urticaria-like rash, fever, arthralgia, and in severe forms sensorineural hearing loss and neurological involvement.
• Sustained IL-1 activity drives high circulating acute-phase reactants including CRP and serum amyloid A, and chronic SAA elevation carries a risk of secondary AA amyloidosis. The dramatic response to IL-1 blockade with anakinra, canakinumab, or rilonacept confirms the pathway.
• Serum is acellular and is the correct matrix for IL-1beta, IL-18, IL-6, CRP, and SAA measurement by immunoassay, and for proteomic work. Inflammasome activation assays require the paired cellular products.

Donor Stratification Available

• Clinical phenotype: FCAS, Muckle-Wells syndrome, or NOMID/CINCA
• NLRP3 genotype where genetic testing is documented, including somatic mosaicism cases
• Disease state at collection: active flare versus inter-flare or controlled disease
• Treatment status: IL-1 blockade-naive versus anakinra, canakinumab, or rilonacept treated
• Organ involvement: sensorineural hearing loss, ocular involvement, or documented AA amyloidosis
• Age band, including paediatric versus adult donors, and cold-exposure trigger history
• Matched healthy controls available

Product Features

• Collected from clinically confirmed cryopyrin-associated periodic syndrome donors
• Processed and frozen within 24 hours of collection
• Research Use Only (RUO)
• IRB-approved protocols with documented consent
• Standard and custom aliquot volumes available

De-identified Donor Data

• Diagnosis confirmation, including genetic confirmation where available
• Age, sex assigned at birth, and ethnicity
• Donor-reported medical history and comorbidities
• CAPS phenotype, flare status, and IL-1 blockade treatment history where documented
• Additional CAPS-specific metadata available on request

Applications

• IL-1beta and IL-18 quantification as direct readouts of inflammasome activity
• Downstream cytokine profiling, including IL-6, TNF, and IL-1 receptor antagonist
• Acute-phase protein measurement, including CRP and serum amyloid A for amyloidosis risk research
• Pharmacodynamic and target-engagement assay development for IL-1-directed biologics
• Flare versus inter-flare biomarker discovery and disease-activity index development
• Untargeted serum proteomics and metabolomics of chronic innate inflammation
• Comparative studies against other monogenic autoinflammatory syndromes and against polygenic inflammatory disease
• Diagnostic assay development, reference-range establishment, and platform bridging for rare-disease panels

Other Cryopyrin-Associated Periodic Syndrome Specimen Types

• Cryopyrin-Associated Periodic Syndrome Plasma
• Cryopyrin-Associated Periodic Syndrome Whole Blood
• Cryopyrin-Associated Periodic Syndrome PBMC

Compliance and Quality Assurance

• IRB-approved and HIPAA-compliant protocols
• Electronic informed consent with 21 CFR Part 11-compliant e-signatures
• Documentation support available for regulatory review

Ordering & Customization

Cryopyrin-associated periodic syndrome serum is available in standard and custom volumes. For pricing, international orders, or documentation requirements, please email learnmore@sanguinebio.com to ensure compatibility with your country’s regulations. To compare donor availability across conditions, visit our human serum page.