TABLE 2.
Presumed functions, N-terminal splicing variants and major phenotypes in mouse models.
Myosin | Presumed functions in the inner ear | N-terminal splicing variants | Major phenotypes in knockout mice | References |
---|---|---|---|---|
MYO1A | Unclear | Not found yet | Knockout mice show no overt phenotypes | Stauffer et al. (2005), Tyska et al. (2005), Solanki et al. (2021) |
MYO1C | Adaptation of MET channels | Not found yet | Homozygous knockout is associated with visual impairment. Hearing loss has not been reported in peer-reviewed journals | Stauffer et al. (2005), Solanki et al. (2021) |
MYO1F | Unclear | Not found yet | Knockout mice show impaired immune responses, but hearing loss is not reported | Kim et al. (2006) |
MYO3A | Transports factors to elongate the F-actin core | Not found yet | Double knockout of Myo3a and Myo3b results in profound deafness and a dysmorphic staircase architecture of stereocilia | Salles et al. (2009), Lelli et al. (2016) |
MYO6 | Tethers plasma membrane to the F-actin core, keeps stereocilia in place and mediates vesicle transport including endocytosis | Not found yet | Loss of MYO6 function results in profound hearing loss and stereocilia bifurcated or fused with each other | Avraham et al. (1995), Avraham et al. (1997), Self et al. (1999), De La Cruz et al. (2001), Melchionda et al. (2001), Ahmed et al. (2003a), Goodyear et al. (2003), Morris et al. (2003), Sakaguchi et al. (2008) |
MYO7A | Helps to form tip-link and MET channel complexes and to tether ankle links during development | MYO7A-C, MYO7A-S | Mice lacking the MYO7A function (e.g., Shaker-1 mice) develop severely deformed stereocilia and show profound hearing loss | Weil et al. (1995), Liu et al. (1997), Weil et al. (1997), Self et al. (1998), Udovichenko et al. (2002), Watanabe et al. (2006), Lefevre et al. (2008), Grillet et al. (2009), Dionne et al. (2018), Jaiganesh et al. (2018) |
MYO15A | Transport factors to elongate the F-actin core, nucleates actin monomers and maintains the length of mechanotransducing stereocilia | MYO15A-1, MYO15A-2, MYO15A-3 | Mice lacking the MYO15A function (e.g., Shaker-2 mice) show short stereocilia and profound hearing loss | Wang et al. (1998), Beyer et al. (2000), Belyantseva et al. (2003), Belyantseva et al. (2005), Mogensen et al. (2007), Zampini et al. (2011), Bird et al. (2014), Fang et al. (2015), Jiang et al. (2020), Moreland et al. (2021) |
MYH9 | Unclear | Not found yet | Myh9-null mice are embryonic lethal. Mice with mutant Myh9 show a phenotype resembling human MYH9-related disease including platelet dysfunction and mild hearing loss | Lalwani et al. (2000), Wang et al. (2000), Mhatre et al. (2007), Yengo et al. (2012), Zhang et al. (2012) |
MYH14 | Unclear | Not found yet | Myh14-null mice are susceptible to noise-induced hearing loss | Donaudy et al. (2004), Heissler and Manstein (2011), Yengo et al. (2012), Fu et al. (2016) |