{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Fleming SM"],"funding":["UC Gardner Family Center for Parkinson’s Disease and Movement Disorders","NINDS NIH HHS","National Institutes of Health","University of Cincinnati Neuroscience Institute"],"pagination":["256-266"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC10178982"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["64"],"pubmed_abstract":["Loss of function mutations in the P<sub>5</sub>-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 region"],"journal":["Neurotoxicology"],"pubmed_title":["The effect of manganese exposure in Atp13a2-deficient mice."],"pmcid":["PMC10178982"],"funding_grant_id":["NS070268","R15 NS070268","R21 NS077022","NS077022"],"pubmed_authors":["Schultheis PJ","Santiago NA","Lemkuhl A","Liou B","Shull GE","Pamphile S","Sun Y","Ekhator OR","Linn SC","Aga DS","Holden JG","Fleming SM","Mullin EJ","Roth JA","Karkare S"],"additional_accession":[]},"is_claimable":false,"name":"The effect of manganese exposure in Atp13a2-deficient mice.","description":"Loss of function mutations in the P<sub>5</sub>-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 region","dates":{"release":"2018-01-01T00:00:00Z","publication":"2018 Jan","modification":"2025-04-26T14:23:46.466Z","creation":"2025-04-06T14:36:49.826Z"},"accession":"S-EPMC10178982","cross_references":{"pubmed":["28595912"],"doi":["10.1016/j.neuro.2017.06.005"]}}