Cochlear molecules and hereditary deafness
by
Yan D, Liu XZ.
Department of Otolaryngology,
Miller School of Medicine,
University of Miami,
Miami, FL 33136, USA.
Front Biosci. 2008 May 1;13:4972-83.

ABSTRACT

Remarkable progress has been made in the past decade in identifying genes involved with deafness in man and mouse. The identification of these genes and functional analysis of the proteins they encode are paving the way towards a better understanding of the physiology and pathophysiology of the auditory system. Given the complexity of auditory transduction and diversity of cochlear structures, it is not surprising that an estimate of at least 1 percent of human protein-coding genes are involved in perception of sound. Over 400 distinct syndromes of which hearing loss is a component have been reported (www.ncbi.nlm.nih.gov/omim). Approximately 113 loci for monogenic disorders for which hearing loss is the only manifestation and therefore is nonsyndromic, have been mapped to the human genome (http:webhost.ua.ac.be/hhh/). As of August 2007, there are approximately 46 genes identified from these loci. Here, we review some of the major advances in our knowledge of auditory function within an evolving understanding of the structure and regulation of the machinery of hearing.

Deafness
Biohappiness
Eugenics talk
Reprogenetics
'Designer babies'
Private eugenics
The Nazi Doctors
Depression genetics
Eugenics before Galton
Scandanavian eugenics
Human self-domestication
Selecting potential children
Preimplantation genetic diagnosis
5-HTTPR polymorphism/depression
Francis Galton and contemporary eugenics
Gene therapy and performance enhancement
The commercialisation of pre-natal enhancement
Transhumanism (H+): toward a Brave New World?
Choosing disability: PGD and 'negative enhancement'

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