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Journal of Neurology Neurosurgery and Psychiatry 2004;75:765-769
© 2004 BMJ Publishing Group Ltd


PAPER

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

T Kanda, Y Numata, H Mizusawa

Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

Correspondence to:
Correspondence to:
Dr T Kanda
Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan; t-kanda.nuro{at}tmd.ac.jp

Objectives: To clarify the dynamics of molecules composing the blood–nerve barrier (BNB) in inflammatory neuropathies.

Methods: The expression of four tight junction (TJ) 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 TJ molecules may suggest new treatments based on properties of the BNB.


Keywords: claudin-1; claudin-5; occluding; ZO-1; chronic inflammatory demyelinating polyneuropathy; blood–nerve barrier; sural nerve

Abbreviations: BBB, blood–brain barrier; BMEC, brain microvascular endothelial cell; BNB, blood–nerve barrier; CNS, central nervous system; CIDP, chronic inflammatory demyelinating polyneuropathy; PBS, phosphate buffered saline; PNS, peripheral nervous system; TJ, tight junction; VEGF, vascular endothelial growth factor




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