Behavioral and neurochemical evaluation of a transgenic mouse model of Lesch-Nyhan syndrome

Journal of the Neurological Sciences

Volumn 86, Issue 2-3, 1988, Pages 203-213

  Finger, S ^a   Heavens, R P ^c   Sirinathsinghji, D J S ^c   Kuehn, M R ^b   Dunnett, S B ^a  

^a UK   (United Kingdom)

^b UNIVERSITY OF CAMBRIDGE   (United Kingdom)

^c BABRAHAM INSTITUTE   (United Kingdom)

Author keywords

Apomorphine; Basal ganglia; Dopamine; Genetics; Hypoxanthine guanine phosphoribosyltransferase; Lesch Nyhan syndrome; Motor systems; Mutation; Purines; Supersensitivity

Indexed keywords

APOMORPHINE; DOPAMINE RECEPTOR; HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE;

ANIMAL CELL; ANIMAL EXPERIMENT; BASAL GANGLION; LESCH NYHAN SYNDROME; MOTOR SYSTEM; MOUSE; MUTANT; NONHUMAN; PRIORITY JOURNAL; SUPERSENSITIVITY;

ANIMAL; APOMORPHINE; AVOIDANCE LEARNING; BIOGENIC AMINES; BRAIN CHEMISTRY; DISEASE MODELS, ANIMAL; HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE; LESCH-NYHAN SYNDROME; MICE; MICE, TRANSGENIC; MOTOR ACTIVITY; MUTATION; REFERENCE VALUES; REFLEX; SUPPORT, NON-U.S. GOV'T; SWIMMING;

EID: 0023679928     PISSN: 0022510X     EISSN: None     Source Type: Journal    
DOI: 10.1016/0022-510X(88)90099-8     Document Type: Article

References (30)

1. Aspects of purine metabolism
2. Neonatal 6-hydroxydopamine treatment: model of susceptability for self-mutilation in the Lesch-Nyhan syndrome
3. Behavioral differences between neonatal and adult 6-hydroxydopamine treated rats to dopamine antagonists: relevance to neurological symptoms in clinical syndromes with reduced brain dopamine
4. Evidence that D-1 receptors contribute to the supersensitive behavioral responses induce by l-dihydroxyphenylalanine in rats treated with 6-hydroxydopamine
5. Neurobiology of D-1 dopamine receptors after neonatal 6-OHDA treatment: relevance to Lesch-Nyhan disease
6. Lesch-Nyhan disease: clinical experience with nineteen patients
7. Transplantation of embryonic ventral forebrain neurons to the neocortex of rats with lesions of nucleus basalis magnocellularis. II. Sensorimotor and learning impairments
8. Self-mutilation in Lesch-Nyhan disease is caused by dopaminergic denervation
9. Dopamine agonist induced compulsive biting behavior in monkeys: animal model for Lesch-Nyhan syndrome
10. Possible involvement of central dopamine receptors in compulsive self-mutilative behavior
11. Circling behavior in rats with partial, unilateral, nigro-striatal lesions: effects of amphetamine, apomorphine and DOPA
12. 3H]spiroperidol binding and rotational behavior in unilaterally 6-hydroxydopamine-lesioned rats
13. HPRT-deficient (Lesch-Nyhan) mouse embryos derived from germline colonization by cultured cells
14. Parkinson's disease
15. Hypoxanthine-guanine phosphoribosyl-transferase deficiency in the Lesch-Nyhan syndrome and gout
16. Clinical syndromes associated with hypoxanthine-guanine phosphoribosyl-transferase deficiency
17. A potential animal model for Lesch-Nyhan syndrome through introduction of HPRT mutations into mice
18. A familial disorder of uric acid metabolism and central nervous system function
19. Biochemical analysis of dysfunction of brain neurotransmitters in the Lesch-Nyhan syndrome
20. The mysterious motor function of the basal ganglia: The Robert Wartenberg Lecture
21. Success and problems of long-term levodopa therapy in Parkinson's disease
22. Somatosensory inattention after dopamine-depleting intracerebral 6-OHDA injections: spontaneous recovery and pharmacological control
23. Application of high performance liquid chromatography with electrochemical detection to neurochemical analysis: measurement of catecholamines, serotonin and metabolites in the rat brain
24. Salvage of circulating hypoxathine by tissues of the mouse
25. Introduction: clinical and genetic features
26. Summary of clinical features
27. Behavior in the Lesch-Nyhan syndrome
28. An enzyme defect associated with a sex-linked human neurological disorder and excessive purine synthesis
29. Lesch-Nyhan syndrome: CSF neurotransmitter abnormalities
30. Apparent sprouting of striatal serotonergic terminals after dopamine-depleting brain lesions