Motor Performances of Spontaneous and Genetically Modified Mutants with Cerebellar Atrophy

Cerebellum. 2019 Jun;18(3):615-634. doi: 10.1007/s12311-019-01017-5.

Abstract

Chance discovery of spontaneous mutants with atrophy of the cerebellar cortex has unearthed genes involved in optimizing motor coordination. Rotorod, stationary beam, and suspended wire tests are useful in delineating behavioral phenotypes of spontaneous mutants with cerebellar atrophy such as Grid2Lc, Grid2ho, Rorasg, Agtpbp1pcd, Relnrl, and Dab1scm. Likewise, transgenic or null mutants serving as experimental models of spinocerebellar ataxia (SCA) are phenotyped with the same tests. Among experimental models of autosomal dominant SCA, rotorod deficits were reported in SCA1 to 3, SCA5 to 8, SCA14, SCA17, and SCA27 and stationary beam deficits in SCA1 to 3, SCA5, SCA6, SCA13, SCA17, and SCA27. Beam tests are sensitive to experimental therapies of various kinds including molecules affecting glutamate signaling, mesenchymal stem cells, anti-oligomer antibodies, lentiviral vectors carrying genes, interfering RNAs, or neurotrophic factors, and interbreeding with other mutants.

Keywords: Cerebellum; Purkinje cells; Rotorod; Spinocerebellar ataxia; Stationary beam; Wire suspension.

Publication types

  • Review

MeSH terms

  • Animals
  • Disease Models, Animal*
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Motor Activity / genetics*
  • Mutation
  • Spinocerebellar Ataxias / genetics*