Chronic inflammatory demyelinating polyneuropathy: decreased claudin-5 and relocated ZO-1

Kanda, T.; Numata, Y.; Mizusawa, H.
May 2004
Journal of Neurology, Neurosurgery & Psychiatry;May2004, Vol. 75 Issue 5, p765
Academic Journal
Objectives: To clarify the dynamics of molecules composing the blood-nerve barrier (BNB) in inflammatory neuropathies. Methods: The expression of four tight junction (Ti) proteins-claudin-1, claudin-5, occludin, and ZO-1 - was analysed immunohistochemically in sural nerve biopsy specimens obtained from patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Results: Claudin-1 was detected only in perineurial cells, whereas claudin-5 was present in endothelial cells, irrespective of vessel location or size. Occludin and ZO-1 were found in perineurial cells, in addition to some epineurial and endoneurial endothelial cells. In CIDP, percentages of endoneurial small vessels immunoreactive for claudin-5 were significantly decreased, as were ZO-1 immunoreactive endoneurial small vessels, with staining localised to interfaces between cells. Claudin-1 and occludin immunoreactivity did not differ appreciably between the neuropathies examined. Conclusions: The downregulation of claudin-5 and altered localisation of ZO-1 seen in CIDP specimens may indicate that BNB derangement occurs in inflammatory neuropathies. Further investigation of Ti molecules may suggest new treatments based on properties of the BNB.


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