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Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation
Ist Teil von
Pflügers Archiv, 2015-01, Vol.467 (1), p.85-94
Ort / Verlag
Berlin/Heidelberg: Springer Berlin Heidelberg
Erscheinungsjahr
2015
Quelle
MEDLINE
Beschreibungen/Notizen
Mutations of the transmembrane channel-like 1 (
TMC1
) gene can cause dominant and recessive forms of deafness in humans and mice.
TMC1
is one of eight mammalian
TMC
genes of unknown function. The multi-pass transmembrane topologic structure of the proteins they encode suggests roles as a receptor, transporter, channel, or pump.
Tmc1
and the closely related
Tmc2
gene are expressed in neurosensory hair cells of the auditory and vestibular end organs of the mouse inner ear. Recent studies have demonstrated that
Tmc1
and
Tmc2
are specifically required for mechanoelectrical transduction in hair cells. The exact role of these proteins in mechanoelectrical transduction is unknown. TMC1 and TMC2 are viable candidates for the mechanoelectrical transduction channel of hair cells, whose component molecules have eluded identification for over 30 years. We expect that studies of TMC proteins will yield insights into molecular components and mechanisms of mechanosensation in auditory and vestibular hair cells, as well as in other tissues and organs.