Journal article

Resolving pathobiological mechanisms relating to Huntington disease: Gait, balance, and involuntary movements in mice with targeted ablation of striatal D1 dopamine receptor cells

Hyun Ah Kim, Luning Jiang, Heather Madsen, Clare L Parish, Jim Massalas, Arthur Smardencas, Claire O'Leary, Ilse Gantois, Colm O'Tuathaigh, John L Waddington, Michelle E Ehrlich, Andrew J Lawrence, John Drago

NEUROBIOLOGY OF DISEASE | ACADEMIC PRESS INC ELSEVIER SCIENCE | Published : 2014

Abstract

Progressive cell loss is observed in the striatum, cerebral cortex, thalamus, hypothalamus, subthalamic nucleus and hippocampus in Huntington disease. In the striatum, dopamine-responsive medium spiny neurons are preferentially lost. Clinical features include involuntary movements, gait and orofacial impairments in addition to cognitive deficits and psychosis, anxiety and mood disorders. We utilized the Cre-LoxP system to generate mutant mice with selective postnatal ablation of D1 dopamine receptor-expressing striatal neurons to determine which elements of the complex Huntington disease phenotype relate to loss of this neuronal subpopulation. Mutant mice had reduced body weight, locomotor s..

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