Mouse Genome Informatics
ht
    Tectatm5.1Gpr/Tecta+
involves: 129S/SvEv
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hearing/vestibular/ear
• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions
• hump-backed tectorial membrane with reduced extent of the limbal zone and medially displaced marginal band
• Kimura's membrane is delaminated and fenestrated, and the Hensen's stripe is no longer attached directly to the tectorial membrane
• most apparent changes are in the cross-sectional profile and the extent of limbal attachment
• covernet fibrils are reduced in numbers, more convoluted and run both longitudinally and radially across the surface of the tectorial membrane compared to in wild-type mice
• covernet fibrils in the sulcal and more medial regions are less tightly associated with the main body of the tectorial membrane
• the striated-sheet matrix is restricted to the lateral part of the tectorial membrane
• the dense matrix is mostly missing from the severely reduced limbal zone
• collagen fibrils erupt form the upper surface of the tectorial membrane in the inner sulcal region and occasionally extend out from the lower surface in the sulcal region immediately adjacent to the limbal attachment point
• more severe hearing loss than in Tectatm3.1Gpr or Tectatm4.1Gpr heterozygotes
• however, increase is stable with time

behavior/neurological
• preceded by wild running

nervous system
• preceded by wild running
• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Dominant 12; DFNA12 601543 J:203482