The Journal of Neuroscience, May 9, 2007, ():
Selective Cochlear Degeneration in Mice Lacking the F-Box Protein, Fbx2, a Glycoprotein-Specific Ubiquitin Ligase Subunit
J. Neurosci. Nelson et al.
27: 5163
Supplemental Data
Files in this Data Supplement:
- supplemental material
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Supplemental Figure 1: Fbx2 and Skp1 expression in wild-type and Fbxo2-/- mice. Coimmunofluorescence staining for Fbx2 and Skp1 in cochlea using confocal microscopy. . Merged images reveal coincident cellular expression of Fbx2 and Skp1 in wild-type mice.
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Supplemental Figure 2: Normal Fbxo2-/- hearing at 1 month of age. Representative Auditory-evoked Brainstem Response (ABR) waveforms from 1 month old Fbxo2 wild-type (WT) and knockout (KO) mice littermates each with thresholds of 15 dB. Threshold levels in decibels (dB) are indicated.
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Supplemental Figure 3: Cochlear degeneration in Fbxo2-/- mice. Shown here are wider ranging views of mid-modiolus cochlear sections in figure 4, highlighting the spiral ganglion neurodegeneration that occurs in Fbxo2-/- mice.
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Supplemental Figure 4: FBXO2 and SKP1 as potential deafness genes. Ideogram of human chromosome 1 (a), and human chromosome 5 (b). Non-syndromic deafness genes are shown along with the location of FBXO2 at 1p36.22 and SKP1 to 5q31, respectively. Known syndromic loci are indicated by an asterisk.
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Supplemental Figure 5: Known FBXO2 small nucleotide polymorphisms (SNPs) and exon junctions. mRNA and protein sequence for human Fbx2. The N-terminal PEST domain (grey), the F-box domain (light grey), and the FBA domain (black) are designated. Brackets are placed around each of the 6 exons of FBXO2. Boxes designate the currently known SNPs for FBXO2 (K118T and R121S).
