Lack of Rbl1-p107 Effects on Cell Proliferation and Maturation in the Inner EarReportar como inadecuado

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Loss of postnatal mammalian auditory hair cells HCs is irreversible. Earlier studies have highlighted the importance of the Retinoblastoma family of proteins pRBs i.e., Rb1, Rbl1-p107, and Rbl2-p130 in the auditory cells’ proliferation and emphasized our lack of information on their specific roles in the auditory system. We have previously demonstrated that lack of Rbl2-p130 moderately affects HCs’ and supporting cells’ SCs proliferation. Here, we present evidence supporting multiple roles for Rbl1-p107 inthe developing and mature mouse organ of Corti OC. Like other pRBs, Rbl1-p107 is expressed in the OC, particularly in the Hensen’s and Deiters’ cells. Moreover, Rbl1-p107 impacts maturation and postmitotic quiescence of HCs and SCs, as evidenced by enhanced numbers of these cells and the presence of dividing cells in the postnatal Rbl1-p107---OC. These findings were further supported by microarray and bioinformatics analyses, suggesting downregulation of several bHLH molecules, as well as activation of the Notch-Hes-Hey signaling pathway in homozygous Rbl1-p107 mutant mice. Physiological assessments and detection of ectopic HC marker expression in postnatal spiral ganglion neurons SGNs provided evidence for incomplete cell maturation and differentiation in Rbl1-p107﹣-﹣OC. Collectively, the present study highlights an important role for Rbl1-p107 inOC cell differentiation and maturation, which is distinct from other pRBs.


Rbl1-p107; Inner Ear; Proliferation; Differentiation; Hair Cells; Supporting Cells

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S. Rocha-Sanchez, L. Scheetz, S. Siddiqi, M. Weston, L. Smith, K. Dempsey, H. Ali, J. McGee and E. Walsh -Lack of Rbl1-p107 Effects on Cell Proliferation and Maturation in the Inner Ear,- Journal of Behavioral and Brain Science, Vol. 3 No. 7, 2013, pp. 534-555. doi: 10.4236-jbbs.2013.37056.

Autor: Sonia M. Rocha-Sanchez, Laura R. Scheetz, Sabrina Siddiqi, Michael W. Weston, Lynette M. Smith, Kate Dempsey, Hesham Ali, JoAnn M



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