| First Author | Noben-Trauth K | Year | 1997 |
| Journal | Genomics | Volume | 44 |
| Issue | 3 | Pages | 266-72 |
| PubMed ID | 9325047 | Mgi Jnum | J:38429 |
| Mgi Id | MGI:86260 | Doi | 10.1006/geno.1997.4869 |
| Citation | Noben-Trauth K, et al. (1997) mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics 44(3):266-72 |
| abstractText | The deaf waddler (dfw) mutation is a model system to study the biology of neuroepithelial hearing defects in mice. Here we describe the identification and characterization of a new allele of deaf waddler (dfw(2J)) and present evidence for a hearing susceptibility locus (mdfw) that interacts with dfw. We found that CBy-dfw(2J)/dfw(2J) homozygotes exhibit no discernible auditory brainstem responses (ABR) to sound pressure level stimuli up to 100 dB, indicating a profound deafness. Interestingly, the ABR iu CBy-dfw(2J)/+ heterozygotes is also abnormal, showing age-dependent elevated thresholds characteristic of a progressive hearing loss, When outcrossed onto the CAST/Ei strain, only 24% of the F2 CBy/CAST-dfw(2J)/+ heterozygotes displayed increased ABR thresholds, suggesting that a second locus, controlling heaping function in dfw(2J)/+ heterozygotes, was segregating in the CBy/CAST-dfw(2J) intercross. By linkage analysis, we localized this locus (mdfw) to Chromosome 10, between markers D10Mit127 and. D10Mit185, within a 4.0 +/- 1.1 cM genetic interval. All CBy/CAST-dfw(2J)/+ heterozygotes that develop hearing loss are homozygous for the CBy- derived recessive allele (mdfw(c)). In contrast, CBy/CAST- dfw(2J)/+ heterozygotes expressing even a single copy of the CAST/Ei-derived mdfw allele (Mdfw) retain their normal heaping function. Our results reveal an epistatic relationship between the mdfw and the dfw genes and provide a model system to study nonsyndromic hearing loss in mice. (C) 1997 Academic Press. |
