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Published on: June 11, 2017
CFTR-deficient pigs display peripheral nervous system defects at birth
Leah R Reznikov1, Qian Dong, Jeng-Haur Chen
1Department of Internal Medicine, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242, USA.
Summary
Cystic fibrosis transmembrane conductance regulator (CFTR) loss directly impacts the nervous system. Studies in pigs show CFTR gene disruption causes myelin defects and nerve conduction delays, suggesting primary neurological issues in cystic fibrosis.
Area of Science:
- Neuroscience
- Genetics
- Cell Biology
Background:
- Peripheral nervous system abnormalities are observed in cystic fibrosis patients.
- These are often considered secondary to the disease's primary effects.
Purpose of the Study:
- To investigate if cystic fibrosis transmembrane conductance regulator (CFTR) gene disruption directly affects nervous system function.
- To explore the role of CFTR in Schwann cells and its impact on myelin and nerve integrity.
Main Methods:
- Studied newborn pigs with CFTR gene knockout (CFTR-/-).
- Examined CFTR expression and activity in Schwann cells.
- Assessed myelin sheath ultrastructure, gene expression (myelin protein zero), axon density, and nerve conduction velocities (trigeminal, sciatic, vestibulocochlear).
- Utilized auditory brainstem evoked potentials for in vivo nerve conduction assessment.
Main Results:
- Confirmed CFTR expression and activity in Schwann cells.
- Observed ultrastructural myelin sheath abnormalities in CFTR-/- pigs.
- Found increased myelin protein zero transcripts, reduced axon density, and decreased nerve conduction velocities.
- Demonstrated delayed vestibulocochlear nerve conduction via auditory brainstem evoked potentials.
Conclusions:
- Loss of CFTR directly impairs Schwann cell function.
- Nervous system defects in cystic fibrosis may be primary manifestations, not just secondary effects.
- Highlights a direct link between CFTR function and peripheral nervous system health.
