Transmembrane inner ear expressed protein (TMIE) has one transmembrane domain and a signal peptide, which encoded by human TMIE gene. Human TMIE protein shares 92% sequence identity with mouse homolog, and exhibits no significant nucleotide or deduced amoni acid sequence similarity to other gene. TMIE mainly express on the plasma membrane, and luminal surface of sensory hair cells. It serves as a site of interaction for other molecules through its highly charged C-terminal domain. TMIE protein plays an important role in normal hearing and balance.
Basic Information of TMIE | |
Protein Name | Transmembrane inner ear expressed protein |
Gene Name | TMIE |
Aliases | Transmembrane inner ear protein |
Organism | Homo sapiens (Human) |
UniProt ID | Q8NEW7 |
Transmembrane Times | 1 |
Length (aa) | 156 |
Sequence | MAGWPGAGPLCVLGGAALGVCLAGVAGQLVEPSTAPPKPKPPPLTKETVVFWDMRLWHVVGIFSLFVLSIIITLCCVFNCRVPRTRKEIEARYLQRKAAKMYTDKLETVPPLNELTEVPGEDKKKKKKKKKDSVDTVAIKVEEDEKNEAKKKKGEK |
Mutation of TMIE in mouse leads to sensory cell defects in the inner ear of spinner, which is a model of human hearing loss deafness autosomal recessive 6. Scientists have reported that TMIE is important for normal hearing and vestibular. The location on the cellular membrane of TMIE indicates it plays a role in signal transduction in the auditory system, and the presence in the stereocilia suggests it might have effects on maintaining the structure and function of the specialized microvilli. TMIE also serves as an essential component of the hair cell’s mechanotransduction machinery that functionally couples the tip link to the transduction channel.
Fig.1. Schematic representation of TMIE. (Ganapathy, 2014)
This article identified TMPRSS3, TMC1, USHIC, CDH23, and TMIE gene, and concluded that all these five genes mutations made great contribution to non-syndromic hearing loss.
The researchers investigated the role of the TMIE in mechanoelectrical transduction through a line of deaf and uncoordinated zebrafish with defective hair-cell function and found that the TMIE protein was essential for normal hearing and balance.
This article indicated that TMIE was an essential component of the hair cell’s mechanotransduction machinery. And the complex formed by PCDH15, TMHS/LHFPL5, and TMIE was important for regulation of channel properties in different hair cells and along the cochlea’s tonotopic axis.
This article elucidated the mechanistic role of TMIE and how specifically it contributed to mechanoelectrical transduction of inner ear hair cells based on the research in 2014 by Zhao et al.
The researchers developed a stable cell line expressing TMIE and found that it mostly localized on the cellular membrane and to a lesser extent in cytoplasm.
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