Rolling Nagoya

Table 2. cacnala (Cav2.1, a1A) Functional results for mutations associated with tottering, leaner, rolling nagoya and rocker... 

Figure 2. Mutations in the human Cav2.1 (P/Q-type) voltage-gated calcium channel associated with Episodic Ataxia Type-2 (EA2) mutations in the Cav2.1 mouse homolog associated with tottering (tg), leaner (tgla), rolling nagoya (tgrol) and rocker phenotypes...

Mori Y, Wakamori M, Oda S, Fletcher CF, Sekiguchi N, Mori E, Copeland NG, Jenkins NA, Matsushita K, Matsuyama Z, Imoto K (2000) Reduced voltage sensitivity of activation of P/Q-type Ca2+ channels is associated with the ataxic mouse mutation rolling Nagoya (tg(rol)). J Neurosci 20 5654-5662. 

There are also a number of murine models of P/Q-type channelopathies. Leaner, Tottering, rolling Nagoya, and Rocker mice all carry mutations in the Cav2.1 calcium channel ai subunit that result in either the substitution of individual amino acids or premature truncations of the channel protein. These mice tend to show an ataxic phenotype and, in some cases, absence seizures (Lorenzon et al. 1998 Mori et al. 2000 Noebels and Sidman 1979 Zwingman et al. 2001). In addition, several of these mutations cause cerebellar atrophy (Herrup and Wilczynski 1982). When introduced into recombinant channels, these mutations tend to reduce channel activity which is consistent with reduced P/Q-type currents in neurons isolated from these mouse lines. 

Suh YS, Oda S, Kang YH, Kim H, Rhyu IJ (2002) Apoptotic cell death of cerebellar granule cells in rolling mouse Nagoya. Neurosci Lett 325 1—4. 

Muroga T, Adachi K, Konagaya M, Takayanagi T, Sobue I (1982) Effects of thyrotropin releasing hormone on cerebellar mutant mice - a kinesiological comparison between rolling mouse Nagoya, weaver and reeler. Jap J Med 27 101-108. 

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