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 2
Distribution of collagen α6(IV) chain in the adult mouse cochlea.
Sections were immunostained using antibodies against collagen α2(IV) (A, I–K: green) and α6(IV) (B–H: green; I–K: red) chains, and CD31 (G, H: red) for the detection of endothelial cells. Subepithelial BMs and perivascular BMs are indicated by open arrowheads and solid arrowheads in the insets, respectively (G, J, K). (I) The superficial plate and invaginated region of the interdental cells network are indicated by open arrows and solid arrows in insets, respectively. (L) Schematic diagram of the distribution of collagen α6(IV) chain [yellow line: subepithelial BMs; pink line: perivascular BMs]. The negative control was established by immunostaining with only the secondary antibody and no specific reaction was observed (S2 Fig). Nuclei were counterstained with Hoechst 33258 (blue). SLim: spiral limbus; B, basilar membrane; SLig, spiral ligament; SV, stria vascularis; 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. Scale bars in inset = 10 μm.