References
Query Results -- Details
MGI Accession ID: MGI:50371
J Number: J:1847
Other Accession IDs:
Title: Amphetamine-induced behavioral phenotype in a hypoxanthine-guanine phosphoribosyltransferase-deficient mouse model of Lesch-Nyhan syndrome.
Authors: Jinnah HA; Gage FH; Friedmann T
Journal: Behav Neurosci
Volume: 105
Issue: 6
Date: 1991 Dec
Year: 1991
Pages: 1004-12
Review Status: Peer Reviewed
Abstract:
In humans, congenital deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT) results in a disorder known as the Lesch-Nyhan syndrome. Patients with this disorder exhibit a prominent neurobehavioral phenotype that results in part from dysfunction of catecholaminergic systems in the striatum. HPRT-deficient mice produced as animal models for this syndrome curiously exhibit no spontaneous neurobehavioral abnormalities. However, the present study demonstrates that HPRT-deficient mice are more sensitive than their HPRT-normal littermates to the ability of amphetamine to stimulate locomotor or stereotypic behaviors. This behavioral supersensitivity to amphetamine indicates the existence of an underlying subclinical abnormality of catecholaminergic systems in the brains of HPRT-deficient mice, analogous to findings in human Lesch-Nyhan patients.
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