Chronic inflammatory demyelinating polyneuropathy: decreased claudin-5 and relocated ZO-1.
OBJECTIVE
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.
Kanda
T
T
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@tmd.ac.jp
Numata
Y
Y
Mizusawa
H
H
D000293:Adolescent; D000328:Adult; D000888:Antibodies, Anti-Idiotypic / Q000276:immunology; D001706:Biopsy; D002556:Cerebrospinal Fluid Proteins / Q000378:metabolism; D003182:Complement C5 / Q000276:immunology; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008565:Membrane Proteins / Q000235:genetics*; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D017354:Point Mutation / Q000235:genetics; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000235:genetics* / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D013497:Sural Nerve / Q000378:metabolism / Q000473:pathology
D000888:Antibodies, Anti-Idiotypic; D002556:Cerebrospinal Fluid Proteins; D003182:Complement C5; D005732:Gangliosides; D008565:Membrane Proteins