Collagen molecules are major
extracellular matrix proteins involved in the development and support of delicate auditory sensory organs.
Type II collagen is widely distributed within inner ear tissues, while type IX is found only within the labyrinthine membrane and dense fibers of the tectorial membrane. Antibody specific for
type II collagen has been shown to be elevated in some patients with
hearing loss due to several presumably autoimmune illnesses (including
Meniere's disease,
otosclerosis, chronic progressive
sensorineural hearing loss, and
relapsing polychondritis). Purified human type II and IX
collagens and an extract of human cochlear tissue were subjected to isolation by
sodium dodecyl sulfate-
polyacrylamide gel electrophoresis (SDS-PAGE) and transferred to
nitrocellulose. The sera of 21 patients with
inner ear disease were examined for the presence of anticollagen and anticochlear
antibodies; the sera were used to probe Western blots of purified human
collagens II, IX, and XI, and cochlear
protein extract with
peroxidase-conjugated goat anti human polyvalent
immunoglobulin as the second antibody. Anti-
type II collagen antibodies were seen in 12 of 21 (57%) patients, while 13 of 21 (62%) had anti-type IX
antibodies detectable by Western blot. A previously unreported 30 kd (probably noncollagen)
protein was 21 (62%) had anti-type IX
antibodies detectable by Western blot. A previously unreported 30 kd (probably noncollagen)
protein was found by SDS-PAGE of human cochlear
tissue extracts, with 3 patients, all with
Meniere's disease, having antibody activity to this
protein detected by Western blot.(ABSTRACT TRUNCATED AT 250 WORDS)