Human Cystic Fibrosis Peripheral Blood Mononuclear Cells

Description

Human Cystic Fibrosis Peripheral Blood Mononuclear Cells (CF PBMCs) are isolated from peripheral blood of patients with genetically confirmed cystic fibrosis (CF), capturing the systemic inflammatory profile of this multi-organ genetic disorder. These human-derived PBMCs contain immune cell populations (T cells, B cells, and monocytes) that demonstrate CF-specific immune dysregulation, including elevated pro-inflammatory cytokine production (IL-1β, IL-6, IL-8), increased neutrophil extracellular trap (NET) formation, and altered macrophage polarization. The cells exhibit typical mononuclear morphology with intact surface markers and maintain hyperinflammatory responses characteristic of CFTR dysfunction. These PBMCs are particularly valuable for studying the complex interplay between CFTR mutations, chronic infection (particularly P. aeruginosa), and sustained immune activation that drives CF pathophysiology. These PBMCs provide a platform for studying PBMC and inflammation in cystic fibrosis, elucidating the B cell role in cystic fibrosis, enabling detailed PBMC phenotyping cystic fibrosis, monitoring inflammation markers in cystic fibrosis, and investigating adaptive immunity in cystic fibrosis.

Application

• Human Cystic Fibrosis Peripheral Blood Mononuclear Cells can be used to explore the effects of CFTR gene mutations on immune cell function, including pathological processes such as abnormal secretion of inflammatory factors, enhanced oxidative stress, and autophagy dysregulation. These cells provide a valuable model for studying systemic immune disorders in cystic fibrosis.