Ultrastructural, physiological, and molecular defects in the inner ear of a gene-knockout mouse model for autosomal Alport syndrome

D Cosgrove, G Samuelson, DT Meehan, C Miller… - Hearing research, 1998 - Elsevier
The cochleae from a COL4A3-deficient mouse line were examined for defects that might
shed light on the molecular mechanism of otopathology observed in humans with Alport
syndrome. At the light microscopic level no obvious defects were observed.
Immunohistochemical analysis using antibodies specific for the basement membrane
collagen chains revealed the absence of the COL4A3 and COL4A4 chains throughout the
membranous labyrinth. The COL4A5 chain was absent from all cochlear basement …