Mouse Genome Informatics
cx
    Ptstm1Ich/Ptstm1Ich
Tg(DBH-PTS)6Csic/0

involves: 129X1/SvJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• striatal tyrosine hydroxylase expression is markedly reduced in the caudoputamen and in the lateral part of the olfactory tubercle but not in the nucleus accumbens
• loss of tyrosine hydroxylase expression results from progressive postnatal loss of expression and failure to increase expression with maturation
• responses to stimulation with a dopamine agonist suggest dopaminergic hypersensitivity in the striatum particularly in the striosomes

behavior/neurological
• first detected at 2 weeks of age
• in balance beam tests mice are slower and make more foot slips
• results suggest mice display hypokinetic hindlimb dystonia

muscle
• results suggest mice display hypokinetic hindlimb dystonia

Mouse Models of Human Disease
OMIM IDRef(s)
Dystonia, Dopa-Responsive; DRD 128230 J:138968