Neurotransmitter changes in the pathophysiology of Lesch-Nyhan syndrome.
The neurological symptoms of Lesch-Nyhan syndrome (LNS) are assumed to result from the neurotransmitter changes in this disorder. Among them, the dopaminergic system is believed to play a role in the self-injurious behavior through receptor supersensitivity. However, the precise mechanism underlying the dopamine supersensitivity remains unclear. An increased serotonergic action in the striatum may be crucial for the appearance of self-injurious behavior, and pharmacological evidence suggests the efficacy of serotonin agonists/antagonists for the treatment of the self-mutilation in LNS.
Saito
Y
Y
Department of Mental Retardation and Birth Defect Research, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan.
Takashima
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S
D000818:Animals; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000473:pathology* / Q000503:physiopathology*; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D018377:Neurotransmitter Agents / Q000502:physiology*; D011954:Receptors, Dopamine / Q000378:metabolism; D012652:Self Mutilation / Q000503:physiopathology*; D013569:Synapses / Q000378:metabolism / Q000473:pathology
D018377:Neurotransmitter Agents; D011954:Receptors, Dopamine