Cerebellar cell degeneration in the leaner mutant mouse
Reference (26)
- et al.
Regional variation and absence of large neurons in the cerebellum of the staggerer mouse
Brain Res.
(1979) - et al.
Spontaneous polyspike discharges in an epileptic mutant mouse (tottering)
Expl Neurol.
(1979) - et al.
Cerebellar gangliosides and phospholipids in mutant mice with ataxia and epilepsy: the tottering/leaner syndrome
Brain Res.
(1981) - et al.
Fate of presynaptic afferents to Purkinje cells in the adult nervous mutant mouse: a model to study presynaptic stabilization
Brain Res.
(1979) - et al.
Changes in the cerebellar noradrenaline nerve terminals of the neurological murine mutant rolling mouse Nagoya: a histofluorescence analysis
Anat. Human Biol. Neurobiol. Neurophysiol.
(1975) - et al.
Tottering, a neuromuscular mutation in the mouse and its linkage with oligosyndactylism
J. Hered.
(1962) A method to correct adequately for the change in neuronal size when estimating numbers after nerve growth factor treatment
J. Neurocytol.
(1976)A formaldehyde-glutaraldehyde fixative of high osmolarity for use in electron-microscopy
J. Cell Biol.
(1965)Methods for the counting of neurons
On the ontogenetic development of the cerebellum (nuclei, fissures, and cortex) of the rat with special reference to regional variations in corticogenesis
J. Hirnforsch.
(1968)
Cerebellar organization in the light of cerebellar nuclear morphology and cerebellar corticogenesis
Ultrastructural changes in the mitochondria of cerebellar Purkinje cells of nervous mutant mice
J. Cell Biol.
Mutant mouse tottering. Selective increase of locuscoeruleus axons in a defined single locus mutation
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Physiology of Dystonia: Animal Studies
2023, International Review of NeurobiologyUsing the shared genetics of dystonia and ataxia to unravel their pathogenesis
2017, Neuroscience and Biobehavioral ReviewsDystonia and cerebellar degeneration in the leaner mouse mutant
2015, Brain ResearchCitation Excerpt :Different Cacan1a mutations in rodent models cause a similarly broad array of disorders, including generalized dystonia or ataxia, paroxysmal dystonia, epilepsy and migraine (Meier and MacPike, 1971; Doyle et al., 1997; van den Maagdenberg et al., 2004; Raike et al., 2005; Tokuda et al., 2007; Shirley et al., 2008). We selected the leaner mutant not because of its relationship to a variety of different disorders, but because it is known to have severe chronic dystonia (Yoon, 1969; Meier and MacPike, 1971; Doyle et al., 1997) that is associated with abnormal physiological activity and slow degeneration of cerebellar Purkinje neurons (Meier and MacPike, 1971; Herrup and Wilczynski, 1982; Heckroth and Abbott, 1994; Lau et al., 2004; Walter et al., 2006; Ovsepian and Friel, 2008, 2012). Thus it provides an ideal tool to address questions regarding the relationships between dystonia and cerebellar dysfunction.
Differential cerebellar GABA<inf>A</inf> receptor expression in mice with mutations in Ca<inf>V</inf>2.1 (P/Q-type) calcium channels
2015, NeuroscienceCitation Excerpt :Several mouse models exist that carry mutations in the orthologous mouse Cacna1a gene. Most of these mutants, including Tottering (tg), Rolling Nagoya (tgrol) and Leaner (tgla), arose spontaneously and display complex phenotypes of cerebellar ataxia, often paired with absence epilepsy and/or other motor phenotypes such as dyskinesia and dystonia (Green and Sidman, 1962; Oda, 1973; Herrup and Wilczynski, 1982). In addition, transgenic knock-in (KI) mouse models were generated which harbor the human FHM1 missense mutations R192Q (R192Q KI) and S218L (S218L KI) in the Cacna1a gene (van den Maagdenberg et al., 2004, 2010).
Mouse Models of Episodic Ataxia Type 2
2015, Movement Disorders: Genetics and Models: Second Edition