The brains of two patients with Lesch-Nyhan syndrome (LNS) were studied. The concentration of dopamine was decreased in the caudate nucleus of LNS patients. Immunohistochemical methods revealed that the dopamine (DA) D1 and D2 receptor and methionine-enkephalin immunoreactivities (IRs) were increased in the putamen, and less significantly in the caudate nucleus. The D1 and D2 receptor IRs of the cingulate cortex, the tryptophan-hydroxylase IR in the dorsal nucleus of the midbrain, as well as the substance P and methionine-enkephalin IRs of the nociception-conducting structures, including the periaqueductal gray and spinal trigeminal nucleus, were not changed. Tyrosine-hydroxylase IR was not decreased in the substantia nigra of the LNS patients. Therefore, the cause of the decreased dopaminergic activity in LNS may not be involved in the production of tyrosine hydroxylase in the substantia nigra. Developmental abnormalities due to the DA defect at an early age might exist in the postsynaptic structure in the striatum.
Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study. Publishing Authors By Initials
Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study. Journal Published:
PUBLICATION TYPE: Journal Article
Journal: Neuropediatrics
VOLUME: 30
Page Numbers: 66-71
Journal Abbreviation: Neuropediatrics
ISSN: 0174-304X
DAY: 16
MONTH: Apr
YEAR: 1999
Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study. Information
Number of References:
LANGUAGE: eng
NlmUniqueID: 8101187
Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study. Keywords Mesh Terms:
KEYWORDS: Up-Regulation
MESH TERMS: biosynthesis
Chemical & Substance for Abstract: Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study. Information
Substance Name: Tyrosine 3-Monooxygenase
Registry Number: EC 1.14.16.2
Grant and Affiliation Information for Dopamine receptor upregulation in Lesch-Nyhan syndrome: a postmortem study.
AFFILIATION: Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
Country: GERMANY
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MEDLINETA: Neuropediatrics
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