Phenotype Image Detail

Image

Caption

Stereocilia are profoundly disorganized in Eps8l2^tm1Wma/Eps8l2^tm1Wma Eps8^tm1Ppdf/Eps8^tm1Ppdf double knockouts. (A) Low-magnification SEM image of the apical organ of Corti (P65). Note that only a few outer hair cells (OHCs) are present, whereas inner hair cells (IHCs) seem to be less affected. (Scale bar, 20 um.) (B) Detail of an OHC bundle (the one indicated with * in A), showing that the staircase pattern of the stereocilia is relatively flattened, with the tall stereocilia (t) being only slightly taller than the shorter stereocilia (s). The rows of stereocilia are also less precisely organized than in normal hair bundles. (Scale bar, 1 um.) (C) Detail of an IHC bundle showing shorter stereocilia than are observed in normal bundles. Row structure is relatively well preserved in this bundle although there are gaps in places (*). (Scale bar, 2.5 um.) (D) Detail of the bundle shown in C (the area marked by the box) with some evidence of a progression in height from short to tall, but tip links are absent. (Scale bar, 0.5 um.) (E) Example of a more highly disrupted IHC bundle compared with C. Row structure and evidence of height increments are more difficult to discern. Some stereocilia are fused (f). The normal radial excitatory axis is indicated with an arrow. (Scale bar, 2 um.)

Copyright

This image is fromFurness DN, Proc Natl Acad Sci U S A 2013 Aug 20;110(34):13898-903. Copyright2013 National Academy of Sciences, U.S.A. J:200669

Associated Alleles

Symbol	Name
Eps8l2^tm1Wma	EPS8-like 2; targeted mutation 1, Walter Marcotti
Eps8^tm1Ppdf	epidermal growth factor receptor pathway substrate 8; targeted mutation 1, Pier Paolo Di Fiore

Associated Genotypes

Allelic Composition	Genetic Background
Eps8^tm1Ppdf/Eps8^tm1Ppdf Eps8l2^tm1Wma/Eps8l2^tm1Wma	involves: 129P2/OlaHsd * C57BL/6J