The Ups and Downs of Retinoic Acid Signaling in Early Inner Ear Development
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چکیده
Deficiency in vitamin A during human development can perturb a number of developmental processes, including cell proliferation, migration, and differentiation, and lead to congenital malformations. There is no de novo synthesis of vitamin A in the human embryo, thus in utero developing tissues are reliant on maternal retinol, which is converted intracellularly into a biologically active derivative of vitamin A, Retinoic Acid (RA). Deficiency in RA affects a number of ectodermally-derived organs [1], including the inner ear. Much of what is known about the requirement for RA during inner ear development has been elucidated through vitamin A deficiency studies [2,3] and analyses of double mutant mice deficient in RA receptors (RARα/ RARγ) [4], in which a pronounced inner ear phenotype is manifested by a marked hypoplasia of the otic vesicle and arrest of development at a rudimentary stage. The inner ear is thus an excellent model in which to investigate the developmental anomalies resulting from deviations in RA concentrations. However, it is not yet fully clear as to what local sources of RA affect inner ear development.
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تاریخ انتشار 2013