MYO7A is required for the functional integrity of the ... - PNAS
Jan 2, 2025 · Here, we show that MYO7A has a distinct role in hair cells, being crucial for the structural integrity of hair bundles. Postnatal deletion of Myo7a leads to 87 to 96% reduction in MYO7A from hair cells by postnatal day 20 (P20), without
Myosin-VIIa is expressed in multiple isoforms and essential for ...
Apr 29, 2020 · MYO7A is proposed to function as a motor that tensions the hair cell mechanotransduction (MET) complex, but conclusive evidence is lacking. Here we report that multiple MYO7A isoforms are...
A Novel Allele of Myosin VIIa Reveals a Critical Function for the C ...
In the inner ear, MYO7A is expressed in mechanosensory hair cells and is required for hair bundle morphogenesis and mechanotransduction (Self et al., 1998; Kros et al., 2002).
Myosin-VIIa is expressed in multiple isoforms and essential for ...
Apr 29, 2020 · Mature IHCs of Myo7a-ΔC mice degenerate over time, giving rise to progressive hearing loss. Taken together, our study reveals an unexpected isoform diversity of MYO7A expression in the cochlea and highlights MYO7A's essential …
MYO7A is required for the functional integrity of the ... - PubMed
Jan 7, 2025 · Here, we show that MYO7A has a distinct role in hair cells, being crucial for the structural integrity of hair bundles. Postnatal deletion of Myo7a leads to 87 to 96% reduction in MYO7A from hair cells by postnatal day 20 (P20), without affecting hearing function.
Rare coding variants involving MYO7A and other genes encoding ...
Sep 15, 2021 · Our findings reveal a co-segregation of rare variants in the MYO7A gene and other structural myosin VIIA binding proteins involved in the tip and ankle links of the hair cell stereocilia. We suggest that recessive digenic inheritance involving these genes could affect the ultrastructure of the stereocilia links in familial MD.
Dual-AAV vector-mediated expression of MYO7A improves …
We observed that shaker-1 mice treated with these dual-AAV vectors had improved cochlear hair cell survival, and many of the surviving hair cells expressed MYO7A. This contrasts with the untreated shaker-1 mice that have severe cochlear hair cell degeneration.
Functional Role of Class III Myosins in Hair Cells - PMC
Feb 25, 2021 · Actin-based myosin motors have been shown to play crucial roles in the development and function of the stereocilia of auditory and vestibular inner ear hair cells. Hair cells can contain hundreds of stereocilia, which rely on myosin motors to elongate, organize, and stabilize their structure.
MYO7A - an overview | ScienceDirect Topics
Myosin VI is expressed in inner and outer hair cells of the sensory epithelium, specifically at the base of the stereocilia, and has a key role in cell motility.
In vivo AAV9- Myo7a gene rescue restores hearing and …
Dec 6, 2024 · In the mammalian cochlea, sensory hair cells are crucial for the transduction of acoustic stimuli into electrical signals, which are then relayed to the central auditory pathway via spiral ganglion neuron (SGN) afferent dendrites.