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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2010-12-17
pubmed:abstractText
The Na(+)/K(+)-ATPase1 alpha subunit 3 (ATP1?(3)) is one of many essential components that maintain the sodium and potassium gradients across the plasma membrane in animal cells. Mutations in the ATP1A3 gene cause rapid-onset of dystonia parkinsonism (RDP), a rare movement disorder characterized by sudden onset of dystonic spasms and slowness of movement. To achieve a better understanding of the pathophysiology of the disease, we used immunohistochemical approaches to describe the regional and cellular distribution of ATP1?(3) in the adult mouse brain. Our results show that localization of ATP1?(3) is restricted to neurons, and it is expressed mostly in projections (fibers and punctuates), but cell body expression is also observed. We found high expression of ATP1?(3) in GABAergic neurons in all nuclei of the basal ganglia (striatum, globus pallidus, subthalamic nucleus, and substantia nigra), which is a key circuitry in the fine movement control. Several thalamic nuclei structures harboring connections to and from the cortex expressed high levels of the ATP1?(3) isoform. Other structures with high expression of ATP1?(3) included cerebellum, red nucleus, and several areas of the pons (reticulotegmental nucleus of pons). We also found high expression of ATP1?(3) in projections and cell bodies in hippocampus; most of these ATP1?(3)-positive cell bodies showed colocalization to GABAergic neurons. ATP1?(3) expression was not significant in the dopaminergic cells of substantia nigra. In conclusion, and based on our data, ATP1?(3) is widely expressed in neuronal populations but mainly in GABAergic neurons in areas and nuclei related to movement control, in agreement with RDP symptoms.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
1096-9861
pubmed:author
pubmed:copyrightInfo
© 2010 Wiley-Liss, Inc.
pubmed:issnType
Electronic
pubmed:day
1
pubmed:volume
519
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
376-404
pubmed:meshHeading
pubmed:year
2011
pubmed:articleTitle
Distribution of Na/K-ATPase alpha 3 isoform, a sodium-potassium P-type pump associated with rapid-onset of dystonia parkinsonism (RDP) in the adult mouse brain.
pubmed:affiliation
Centre for Membrane Pumps in Cells and Disease-PUMPKIN, Danish National Research Foundation.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't