Brain, Vol 121, Issue 10 1895-1902, Copyright © 1998 by Oxford University Press
CM Gabriel, RA Hughes, SE Moore, KJ Smith and FS Walsh
Two myelin proteins, P2 basic protein and P0 glycoprotein, can induce
experimental autoimmune neuritis (EAN), a model of human inflammatory
neuropathy. We investigated whether peripheral nerve myelin protein-22
(PMP22), the gene for which is duplicated in hereditary motor sensory
neuropathy type la, can also induce EAN. PMP22 cDNA produced by the reverse
transcriptase-polymerase chain reaction from rat sciatic nerve was
expressed in Escherichia coli as a fusion protein with glutathione-
S-transferase (GST). Ten Lewis rats were immunized with purified PMP22
fusion protein (50-100 microg) and eight controls with the same amount of
GST. Two additional animals were immunized with each of two peptides (250
microg) of the human PMP22 extracellular sequences. Animals were examined
daily until 20 days following immunization, when they underwent
neurophysiological examination. A serum sample was then taken, prior to
perfusion with glutaraldehyde and removal of the sciatic nerves and cauda
equina. PMP22-immunized animals developed antibodies to the fusion protein
and five out of 10 developed limp tails. No changes were observed in
controls immunized with GST or in animals immunized with peptide. The mean
compound motor action potentials elicited in the foot muscles by
stimulation of the sciatic nerve at the sciatic notch and of the tibial
nerve at the ankle were significantly reduced in the PMP22-immunized group
(P < 0.05). Spinal roots from the group of animals immunized with PMP22
showed sparse infiltration of mononuclear cells, oedema and demyelination.
PMP22 now deserves consideration as an autoantigen in human acute
inflammatory demyelinating polyradiculoneuropathy.
ARTICLES
Induction of experimental autoimmune neuritis with peripheral myelin protein-22
Department of Neurology, UMDS, Guy's Hospital, London, UK.
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