Miyoshi Takushi, Vishwasrao Harshad D, Belyantseva Inna A, Miyoshi Junko, Sajeevadathan Mrudhula, Ishibashi Yasuko, Adadey Samuel M, Harada Narinobu, Shroff Hari, Friedman Thomas B
Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, MD, USA.
Division of Molecular and Integrative Physiology, Department of Biomedical Sciences, Southern Illinois University School of Medicine, Carbondale, IL, USA.
Nat Commun. 2025 Sep 1;16(1):8149. doi: 10.1038/s41467-025-63102-0.
Stereocilia are F-actin-based cylindrical protrusions on the apical surface of inner ear hair cells that function as biological mechanosensors of sound and acceleration. During stereocilia development, specific unconventional myosins transport proteins and phospholipids as cargo and mediate elongation, differentiation and acquisition of the mechanoelectrical transduction (MET). How unconventional myosins localize themselves and cargo in stereocilia using energy from ATP hydrolysis is only partially understood. Here, we developed STELLA-SPIM microscopy to visualize movement of single myosin molecules in live hair cell stereocilia. STELLA-SPIM demonstrated that MYO7A, a component of MET machinery, shows processive movement toward stereocilia tips when chemically dimerized or constitutively activated by missense mutations disabling tail-mediated autoinhibition. Conversely, MYO7A shows step-wise but not processive movement in stereocilia when its tail is tethered to the plasma membrane or F-actin in the presence of MYO7A interacting partners. We posit that MYO7A dimerizes and moves processively in stereocilia when unleashed from autoinhibition.
静纤毛是内耳毛细胞顶端表面基于丝状肌动蛋白的圆柱形突起,作为声音和加速度的生物机械传感器发挥作用。在静纤毛发育过程中,特定的非常规肌球蛋白将蛋白质和磷脂作为货物运输,并介导静纤毛的伸长、分化以及机械电转导(MET)的获得。目前仅部分了解非常规肌球蛋白如何利用ATP水解产生的能量将自身及货物定位于静纤毛中。在此,我们开发了STELLA-SPIM显微镜,以可视化活毛细胞静纤毛中单个肌球蛋白分子的运动。STELLA-SPIM表明,MET机制的一个组成部分MYO7A在化学二聚化或因错义突变导致尾部介导的自抑制失活而组成性激活时,会向静纤毛尖端进行持续性运动。相反,当MYO7A的尾部在存在MYO7A相互作用伙伴的情况下与质膜或丝状肌动蛋白相连时,MYO7A在静纤毛中表现出逐步运动但非持续性运动。我们推测,当MYO7A从自抑制中释放出来时,它会在静纤毛中发生二聚化并进行持续性运动。