33 research outputs found
Enlargement of GAD-immunopositive terminals in the
Reorganization of cerebellar circuitry due to
specific cell loss in Weaver mutants causes physiological
and morphological alterations in the terminal domains of
the GABAergic cerebellar corticovestibular projections.
In this study sizes of anti-GAD immunopositive
terminals in the dorsal part of the lateral vestibular
nucleus (dLVN) of normal mice and Weaver mutants
were quantified morphometrically. In anti-GADimmunoreacted
material terminal sizes in the dLVN of
Weaver exceed significantly those of coprocessed
wildtypes. This suggests that the cerebellar disturbances
in Weaver predispose the normal synaptic remodelling
observed in wildtypes towards the formation of enlarged
terminals
Reaktionsmuster einzelner Neurone in Geniculatum laterale und visuellen Cortex der Katze bei Reizung mit optokinetischen Streifenmustern
Reaktionsmuster der Neurone im zentralen visuellen System von Fischen, Kaninchen und Katzen auf monoculare und binoculare Lichtreize
Maintained activity in the lateral geniculate body of the cat and the effects of visual deprivation
The ataxia (axJ) mutation causes abnormal GABAA receptor turnover in mice
Ataxia represents a pathological coordination failure that often involves functional disturbances in cerebellar circuits. Purkinje cells (PCs) characterize the only output neurons of the cerebellar cortex and critically participate in regulating motor coordination. Although different genetic mutations are known that cause ataxia, little is known about the underlying cellular mechanisms. Here we show that a mutated ax(J) gene locus, encoding the ubiquitin-specific protease 14 (Usp14), negatively influences synaptic receptor turnover. Ax(J) mouse mutants, characterized by cerebellar ataxia, display both increased GABA(A) receptor (GABA(A)R) levels at PC surface membranes accompanied by enlarged IPSCs. Accordingly, we identify physical interaction of Usp14 and the GABA(A)R alpha1 subunit. Although other currently unknown changes might be involved, our data show that ubiquitin-dependent GABA(A)R turnover at cerebellar synapses contributes to ax(J)-mediated behavioural impairment