Abnormalities of Purkinje Cell Arborization in Brindled Mouse Cerebellum

The study investigated the cerebellum of the hemizygous brindled mouse (MObr/y), a model for Kinky hair disease (KHD) in humans, using the Golgi technique. In 15-day-old MObr/y mice, Purkinje cells exhibited abnormal arborization, including perisomatic dendrite-like processes and poor dendritic tree development, resembling KHD. These abnormalities were not present in control mice after day 12. Treatment with cupric chloride (CuCl2) on postnatal days seven and ten corrected these abnormalities by day 15, although some changes reappeared transiently after day 23 but resolved by day 110. The study suggested that delayed maturation of Purkinje cells in MObr/y mice could be corrected by CuCl2 treatment, and the observed dendritic deformities might be secondary to the loss of other neurons, as other neuronal populations were preserved in the mutant mice.