Pael receptor is involved in dopamine metabolism in the nigrostriatal system.
Neurosci Res
; 59(4): 413-25, 2007 Dec.
Article
em En
| MEDLINE
| ID: mdl-17889953
ABSTRACT
Pael receptor (Pael-R) has been identified as one of the substrates of Parkin, a ubiquitin ligase responsible for autosomal recessive juvenile Parkinsonism (AR-JP). When Parkin is inactivated, unfolded Pael-R accumulates in the endoplasmic reticulum and results in neuronal death by unfolded protein stress, suggesting that Pael-R has an important role in the pathogenesis of AR-JP. Here we report the analyses on Pael-R-deficient (KO) and Pael-R-transgenic (Tg) mice. The striatal dopamine (DA) level of Pael-R KO mice was only 60% of that in normal mice, while in Pael-R Tg mice, striatal 3,4-dihydroxyphenylacetic acid (DOPAC) as well as vesicular DA content increased. Moreover, the nigrostriatal dopaminergic neurons of Pael-R Tg mice are more vulnerable to Parkinson's disease-related neurotoxins while those of Pael-R KO mice are less. These results strongly suggest that the Pael-R signal regulates the amount of DA in the dopaminergic neurons and that excessive Pael-R expression renders dopaminergic neurons susceptible to chronic DA toxicity.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Substância Negra
/
Dopamina
/
Corpo Estriado
/
Receptores Acoplados a Proteínas G
/
Vias Neurais
Limite:
Animals
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Humans
/
Male
Idioma:
En
Ano de publicação:
2007
Tipo de documento:
Article