Lack of collagen α6(IV) chain in mice does not cause severe-to-profound hearing loss or cochlear malformation, a distinct phenotype from nonsyndromic hearing loss with COL4A6 missense mutation
Fig 3
Distribution of collagen α5(IV) chain in the cochlea of WT and Col4a6 KO mice.
(A, D–F) The collagen α5(IV) chain located in the subepithelial BMs, perivascular BMs, and perineural BMs in WT mice (green). (B, G–I) In Col4a6 KO mice, the α5(IV) chain is widely distributed (green). However, weak signals were detected in the perivascular BMs of the spiral limbus (SLim), spiral ligament (SLig), and stria vascularis (SV) (dashed arrows). (C) No immunopositive signal of the collagen α6(IV) chain was present in Col4a6 KO mouse cochlea (green). Nuclei were counterstained with Hoechst 33258 (blue). B, basilar membrane; RM, Reissner’s membrane; NF, neural fasciculus; SG, spiral ganglion; IDC, interdental cells; ISC, inner sulcus cells; OSC, outer sulcus cells; RC, root cells; Cap, capillary. Scale bars = 50 μm.