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[Changes in the granulocyte membrane in mucoviscidosis]
Klinische Padiatrie
|September 1, 1983
Summary
Polymorphonuclear leukocyte (PMN) cytoskeleton defects are common in cystic fibrosis, impacting immune function. These defects, linked to chronic infections, may promote further illness in cystic fibrosis patients.
Area of Science:
- Cell biology
- Immunology
- Biochemistry
Context:
- Polymorphonuclear leukocytes (PMNs) are crucial immune cells.
- Cytoskeleton integrity, comprising microtubule and microfilament systems, is vital for PMN functions.
- Concanavalin A (Con A) receptor complex mobility assays surface distribution patterns, indicating cytoskeleton status.
Purpose:
- To investigate cytoskeleton integrity in PMNs from cystic fibrosis patients.
- To determine if chronic bacterial infections in cystic fibrosis impact PMN cytoskeleton.
- To assess the role of cytoskeleton defects as a potential infection-promoting factor in cystic fibrosis.
Summary:
- 44% of cystic fibrosis PMNs showed intact cytoskeleton compared to 69% of controls, based on Con A distribution.
- Con A capping (microtubule alteration) was observed in 32% of cystic fibrosis PMNs versus 23% of controls.
- Patchy Con A distribution (microtubule and microfilament disruption) was significantly higher in cystic fibrosis PMNs (24%) than controls (8%).
Impact:
- Findings suggest chronic bacterial infections induce PMN cytoskeleton defects in cystic fibrosis.
- These cytoskeleton defects represent a leukocyte-function impairment.
- This impairment may act as an additional factor promoting infection progression in cystic fibrosis.