The non-typeable
Haemophilus influenzae strain DH1 was isolated from a 25 year old malepatient with Fisher syndrome, a postinfe
ctious autoimmune
condition
chara
cterized by the presen
ce ofanti-GQ1b IgG antibodies that target and initiate damage to peripheral nerves. DH1 was found to displayan
chars/alpha.gif" BORDER=0>NeuA
c(2-8)
chars/alpha.gif" BORDER=0>NeuA
c(2-3)
chars/beta2.gif" BORDER=0 ALIGN="middle">Gal bran
ch bound to the tetraheptosyl ba
ckbone
core of its lipooligosa
ccharide (LOS). The novel sialylation pattern was found to be dependent on the a
ctivity of a bifun
ctionalsialyltransferase, Li
c3B, whi
ch
catalyzes the addition of both the terminal and subterminal siali
c a
cidresidues. Patient serum IgGs bind to DH1 LOS, and the rea
ctivity is signifi
cantly influen
ced by the presen
ceof sialylated gly
coforms. The display by DH1, of a surfa
ce gly
can that mimi
cs the terminal trisa
ccharideportion of disialosyl-
containing gangliosides, provides strong eviden
ce for its involvement in thedevelopment of Fisher syndrome.