1
40
3
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.neuro.2017.06.005" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.neuro.2017.06.005</a>
Pages
256–266
Volume
64
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
The effect of manganese exposure in Atp13a2-deficient mice.
Publisher
An entity responsible for making the resource available
Neurotoxicology
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
2018-01
Subject
The topic of the resource
*Alpha-synuclein; *Lipofuscin; *Manganese; *Mice; *Parkinson's disease; *Sensorimotor function
Creator
An entity primarily responsible for making the resource
Fleming Sheila M; Santiago Nicholas A; Mullin Elizabeth J; Pamphile Shanta; Karkare Swagata; Lemkuhl Andrew; Ekhator Osunde R; Linn Stephen C; Holden John G; Aga Diana S; Roth Jerome A; Liou Benjamin; Sun Ying; Shull Gary E; Schultheis Patrick J
Description
An account of the resource
Loss of function mutations in the P5-ATPase ATP13A2 are associated with Kufor-Rakeb Syndrome and Neuronal Ceroid Lipofuscinosis. While the function of ATP13A2 is unclear, in vitro studies suggest it is a lysosomal protein that interacts with the metals manganese (Mn) and zinc and the presynaptic protein alpha-synuclein. Loss of ATP13A2 function in mice causes sensorimotor deficits, enhanced autofluorescent storage material, and accumulation of alpha-synuclein. The present study sought to determine the effect of Mn administration on these same outcomes in ATP13A2-deficient mice. Wildtype and ATP13A2-deficient mice received saline or Mn at 5-9 or 12-19 months for 45days. Sensorimotor function was assessed starting at day 30. Autofluorescence was quantified in multiple brain regions and alpha-synuclein protein levels were determined in the ventral midbrain. Brain Mn, iron, zinc, and copper concentrations were measured in 5-9 month old mice. The results show Mn enhanced sensorimotor function, increased autofluorescence in the substantia nigra, and increased insoluble alpha-synuclein in the ventral midbrain in older ATP13A2-deficient mice. In addition, the Mn regimen used increased Mn concentration in the brain and levels were higher in Mn-treated mutants than controls. These results indicate loss of ATP13A2 function leads to increased sensitivity to Mn in vivo.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.neuro.2017.06.005" target="_blank" rel="noreferrer noopener">10.1016/j.neuro.2017.06.005</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
*Alpha-synuclein
*Lipofuscin
*Manganese
*Mice
*Parkinson's disease
*Sensorimotor function
2018
Aga Diana S
Department of Pharmaceutical Sciences
Ekhator Osunde R
Fleming Sheila M
Holden John G
Karkare Swagata
Lemkuhl Andrew
Linn Stephen C
Liou Benjamin
Mullin Elizabeth J
NEOMED College of Pharmacy
Neurotoxicology
Pamphile Shanta
Roth Jerome A
Santiago Nicholas A
Schultheis Patrick J
Shull Gary E
Sun Ying
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.neuro.2017.06.005" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.neuro.2017.06.005</a>
Rights
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Pages
256-266
Volume
64
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
The effect of manganese exposure in Atp13a2-deficient mice.
Publisher
An entity responsible for making the resource available
Neurotoxicology
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
2018-01
Subject
The topic of the resource
Female; Male; Animals; Mice; *Parkinson's disease; *Alpha-synuclein; Mice; Membrane Proteins/genetics/*metabolism; Motor Activity; *Lipofuscin; *Manganese; *Sensorimotor function; Behavior; Inbred C57BL; Animal; Knockout; Adenosine Triphosphatases/genetics/*metabolism; alpha-Synuclein/metabolism; Brain/*drug effects/*metabolism; Manganese/metabolism/*toxicity
Creator
An entity primarily responsible for making the resource
Fleming Sheila M; Santiago Nicholas A; Mullin Elizabeth J; Pamphile Shanta; Karkare Swagata; Lemkuhl Andrew; Ekhator Osunde R; Linn Stephen C; Holden John G; Aga Diana S; Roth Jerome A; Liou Benjamin; Sun Ying; Shull Gary E; Schultheis Patrick J
Description
An account of the resource
Loss of function mutations in the P5-ATPase ATP13A2 are associated with Kufor-Rakeb Syndrome and Neuronal Ceroid Lipofuscinosis. While the function of ATP13A2 is unclear, in vitro studies suggest it is a lysosomal protein that interacts with the metals manganese (Mn) and zinc and the presynaptic protein alpha-synuclein. Loss of ATP13A2 function in mice causes sensorimotor deficits, enhanced autofluorescent storage material, and accumulation of alpha-synuclein. The present study sought to determine the effect of Mn administration on these same outcomes in ATP13A2-deficient mice. Wildtype and ATP13A2-deficient mice received saline or Mn at 5-9 or 12-19 months for 45days. Sensorimotor function was assessed starting at day 30. Autofluorescence was quantified in multiple brain regions and alpha-synuclein protein levels were determined in the ventral midbrain. Brain Mn, iron, zinc, and copper concentrations were measured in 5-9 month old mice. The results show Mn enhanced sensorimotor function, increased autofluorescence in the substantia nigra, and increased insoluble alpha-synuclein in the ventral midbrain in older ATP13A2-deficient mice. In addition, the Mn regimen used increased Mn concentration in the brain and levels were higher in Mn-treated mutants than controls. These results indicate loss of ATP13A2 function leads to increased sensitivity to Mn in vivo.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.neuro.2017.06.005" target="_blank" rel="noreferrer noopener">10.1016/j.neuro.2017.06.005</a>
*Alpha-synuclein
*Lipofuscin
*Manganese
*Parkinson's disease
*Sensorimotor function
2018
Adenosine Triphosphatases/genetics/*metabolism
Aga Diana S
alpha-Synuclein/metabolism
Animal
Animals
Behavior
Brain/*drug effects/*metabolism
Department of Pharmaceutical Sciences
Ekhator Osunde R
Female
Fleming Sheila M
Holden John G
Inbred C57BL
Karkare Swagata
Knockout
Lemkuhl Andrew
Linn Stephen C
Liou Benjamin
Male
Manganese/metabolism/*toxicity
Membrane Proteins/genetics/*metabolism
Mice
Motor Activity
Mullin Elizabeth J
NEOMED College of Pharmacy
Neurotoxicology
Pamphile Shanta
Roth Jerome A
Santiago Nicholas A
Schultheis Patrick J
Shull Gary E
Sun Ying
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1093/hmg/ddw322" target="_blank" rel="noreferrer noopener">http://doi.org/10.1093/hmg/ddw322</a>
Pages
5126–5141
Issue
23
Volume
25
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.
Publisher
An entity responsible for making the resource available
Human molecular genetics
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
2016-12
Subject
The topic of the resource
Animal; Animals; Calcium Signaling/genetics; Dantrolene/*administration & dosage; Disease Models; Gaucher Disease/*drug therapy/genetics/physiopathology; Humans; Mice; Mitochondria/*drug effects/genetics/pathology; Neurons/drug effects/pathology; Neuroprotective Agents/administration & dosage; Ryanodine Receptor Calcium Release Channel/*genetics/metabolism
Creator
An entity primarily responsible for making the resource
Liou Benjamin; Peng Yanyan; Li Ronghua; Inskeep Venette; Zhang Wujuan; Quinn Brian; Dasgupta Nupur; Blackwood Rachel; Setchell Kenneth D R; Fleming Sheila; Grabowski Gregory A; Marshall John; Sun Ying
Description
An account of the resource
Neuronopathic Gaucher disease (nGD) manifests as severe neurological symptoms in patients with no effective treatment available. Ryanodine receptors (Ryrs) are a family of calcium release channels on intracellular stores. The goal of this study is to determine if Ryrs are potential targets for nGD treatment. A nGD cell model (CBE-N2a) was created by inhibiting acid beta-glucosidase (GCase) in N2a cells with conduritol B epoxide (CBE). Enhanced cytosolic calcium in CBE-N2a cells was blocked by either ryanodine or dantrolene, antagonists of Ryrs and by Genz-161, a glucosylceramide synthase inhibitor, suggesting substrate-mediated
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1093/hmg/ddw322" target="_blank" rel="noreferrer noopener">10.1093/hmg/ddw322</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
Animal
Animals
Blackwood Rachel
Calcium Signaling/genetics
Dantrolene/*administration & dosage
Dasgupta Nupur
Department of Pharmaceutical Sciences
Disease Models
Fleming Sheila
Gaucher Disease/*drug therapy/genetics/physiopathology
Grabowski Gregory A
Human molecular genetics
Humans
Inskeep Venette
Li Ronghua
Liou Benjamin
Marshall John
Mice
Mitochondria/*drug effects/genetics/pathology
NEOMED College of Pharmacy
Neurons/drug effects/pathology
Neuroprotective Agents/administration & dosage
Peng Yanyan
Quinn Brian
Ryanodine Receptor Calcium Release Channel/*genetics/metabolism
Setchell Kenneth D R
Sun Ying
Zhang Wujuan