Neurochemical and Immunocytochemical Studies of Catecholamine System in the Brindled Mouse

The study investigated catecholamine neurons in brindled mottled mice, a model for Kinky hair syndrome, using tyrosine hydroxylase (TH) and dopamine-beta-hydroxylase (DBH) markers. A transient increase in TH-immunoreactive neurons (TH-IN) was observed in the cerebral cortex during the second postnatal week, especially in hemizygous brindled males (MObr/y). These mice also showed a rapid increase of TH-IN in the striatum after postnatal day 11, which was rare in controls. Cupric chloride treatment reduced TH-IN visibility in some MObr/y mice. No differences were found in TH-IN in the substantia nigra and ventral tegmental area between MObr/y and controls. MObr/y mice had a more pronounced superficial plexus of DBH fibers but no DBH neurons in the cerebral cortex or striatum. Neurochemical analysis showed decreased norepinephrine and increased serotonin and its metabolites in MObr/y brains, suggesting abnormal catecholamine neuron development due to copper deficiency.