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. 2013 Apr 23;7:4. doi: 10.3389/fnana.2013.00004

Table 1.

Main mutations affecting cerebellar Purkinje cells (Pc).

Mutation Onset of Pc degeneration End of Pc degeneration Size of Pc degeneration Cerebellar and pre-cerebellar associated cell degenerations Associate neural degenerative changes Motor impairment References
Leaner (tg1a/tg1a)1 P15–40 5, 6 month Alternate Pc bands with bands devoid of Pc2 Golgi neurons3, granule cells3, inferior olivary neurons4 Hippocampus5, 6 Most severe that in other mutations6

  • 1Dickie (1962)

  • 2Heckroth and Abbott (1994)

  • 3Herrup and Wilczynski (1982)

  • 4Zanjani et al. (2004)

  • 5Alonso et al. (2008)

  • 6Grüsser-Cornehls and Bäurle (2001)
Lurcher (Lc/+)1 P3–4 4 month Complete Inferior olivary neurons2, granule cells2 No data available Mild3

  • 1Phillips (1960)

  • 2Caddy and Biscoe (1979)

  • 3Grüsser-Cornehls and Bäurle (2001)
Nervous (nr/nr)1 P23 P50 Alternate Pc bands with bands devoid of Pc2 Inferior olivary neurons3 Retinal photoreceptors4 Mild5

  • 1Sidman and Green (1970)

  • 2Wassef et al. (1987)

  • 3Zanjani et al. (2004)

  • 4Mullen and LaVail (1975)

  • 5Grüsser-Cornehls and Bäurle (2001)
Pogo (pogo/pogo)1 P120 – Vermal Pc2 No data available No data available Mild1, 2

  • 1Lee and Jeong (2009)

  • 2Jeong et al. (2000)
Purkinje cell degeneration (pcd/pcd)1 P15 P45 Complete Inferior olivary neurons2, granule cells3, cerebellar nuclei neurons4 Retinal photoreceptors1, olfactory bulb mitral cells1, thalamic neurons5 Mild6

  • 1Mullen et al. (1976)

  • 2Ghetti et al. (1987)

  • 3, 4Triarhou et al. (1985); Triarhou et al. (1987)

  • 5O'Gorman (1985)

  • 6Grüsser-Cornehls and Bäurle (2001)
Reeler (rl/rl)1 P0 P15 ≤50% with ectopic remaining Pc2 GABAergic interneurons3, granule cells4, unipolar brush cells4 Hippocampus5, neocortex5 Mild

  • 1Falconer (1951)

  • 2Heckroth et al. (1998)

  • 3Takayama (1994)

  • 4Ilijic et al. (2005)

  • 5Park and Tom Curran (2008)
Staggerer (sg/sg)1 P0 P24 75% with ectopic remaining Pc2 All granule cells2, inferior olivary neurons3 Hippocampus4, olfactory bulb5 Most severe than in Lc, nr, or pcd6

  • 1Sidman et al. (1962)

  • 2Sotelo and Changeux (1974a)

  • 3Zanjani et al. (2007)

  • 4Yi et al. (2010)

  • 5Deiss et al. (2001)

  • 6Grüsser-Cornehls and Bäurle (2001)
Tambaleante (tbl/tbl)1 P60 4–6 month Complete1 No data available No data available Mild, like Lc or pcd2, 3

  • 1Wassef et al. (1987)

  • 2Rossi et al. (1995) 3present observations
Weaver (wv/wv)1 P0 P14 25% with great alteration of the dendritic trees of remaining Pc2, 3 Granule cells2, 3 Hippocampus4, substantia nigra pars compacta5 Most severe than in Lc, nr, pcd, or sg6

  • 1Lane (1965)

  • 2Sotelo and Changeux (1974b)

  • 3Sotelo (1980)

  • 4Sekiguchi et al. (1995)

  • 5Schmidt et al. (1982)

  • 6Grüsser-Cornehls and Bäurle (2001)