{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T23:51:01Z","timestamp":1790725861466,"version":"4.1.0"},"reference-count":47,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T00:00:00Z","timestamp":1635724800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T00:00:00Z","timestamp":1635724800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,9,14]],"date-time":"2021-09-14T00:00:00Z","timestamp":1631577600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R37NS096241"],"award-info":[{"award-number":["R37NS096241"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000055","name":"National Institute on Deafness and Other Communication Disorders","doi-asserted-by":"publisher","award":["R01DC06519"],"award-info":[{"award-number":["R01DC06519"]}],"id":[{"id":"10.13039\/100000055","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2021,11]]},"DOI":"10.1016\/j.nbd.2021.105513","type":"journal-article","created":{"date-parts":[[2021,9,15]],"date-time":"2021-09-15T21:44:59Z","timestamp":1631742299000},"page":"105513","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":28,"special_numbering":"C","title":["Heterozygous GBA D409V and ATP13a2 mutations do not exacerbate pathological \u03b1-synuclein spread in the prodromal preformed fibrils model in young mice"],"prefix":"10.1016","volume":"159","author":[{"given":"Michaela E.","family":"Johnson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liza","family":"Bergkvist","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas","family":"Stetzik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer A.","family":"Steiner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lindsay","family":"Meyerdirk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Schulz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Wolfrum","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelvin C.","family":"Luk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel W.","family":"Wesson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dimitri","family":"Krainc","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrik","family":"Brundin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2021.105513_bb0005","doi-asserted-by":"crossref","first-page":"2648","DOI":"10.1093\/brain\/awv179","article-title":"Glucocerebrosidase activity in Parkinson\u2019s disease with and without GBA mutations","volume":"138","author":"Alcalay","year":"2015","journal-title":"Brain."},{"key":"10.1016\/j.nbd.2021.105513_bb0010","doi-asserted-by":"crossref","first-page":"113693","DOI":"10.1016\/j.expneurol.2021.113693","article-title":"An extended release GLP-1 analogue increases \u03b1-synuclein accumulation in a mouse model of prodromal Parkinson\u2019s disease","volume":"341","author":"Bergkvist","year":"2021","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.nbd.2021.105513_bb0015","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aau6870","article-title":"A modulator of wild-type glucocerebrosidase improves pathogenic phenotypes in dopaminergic neuronal models of Parkinson\u2019s disease","volume":"11","author":"Burbulla","year":"2019","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.nbd.2021.105513_bb0020","article-title":"Moving towards a new era of genomics in the neuronal ceroid lipofuscinoses","volume":"1866","author":"Butz","year":"2019","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Basis Dis."},{"key":"10.1016\/j.nbd.2021.105513_bb0025","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1002\/mds.28404","article-title":"Neuroimaging of anxiety in Parkinson\u2019s disease: a systematic review","volume":"36","author":"Carey","year":"2021","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105513_bb0030","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.1038\/ng.3955","article-title":"A meta-analysis of genome-wide association studies identifies 17 new Parkinson\u2019s disease risk loci","volume":"49","author":"Chang","year":"2017","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2021.105513_bib231","article-title":"Neuropathologic findings in a patient with juvenile-onset levodopa responsive parkinsonism due to ATP13A2 mutation","author":"Chien","year":"2021","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2021.105513_bb0035","doi-asserted-by":"crossref","first-page":"1557","DOI":"10.1212\/01.wnl.0000260963.08711.08","article-title":"ATP13A2 missense mutations in juvenile parkinsonism and young onset Parkinson disease","volume":"68","author":"Di Fonzo","year":"2007","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105513_bb0040","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.bbr.2018.01.029","article-title":"Exacerbation of sensorimotor dysfunction in mice deficient in Atp13a2 and overexpressing human wildtype alpha-synuclein","volume":"343","author":"Dirr","year":"2018","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.nbd.2021.105513_bb0045","doi-asserted-by":"crossref","first-page":"3235","DOI":"10.1093\/brain\/awu291","article-title":"Augmentation of phenotype in a transgenic Parkinson mouse heterozygous for a Gaucher mutation","volume":"137","author":"Fishbein","year":"2014","journal-title":"Brain."},{"key":"10.1016\/j.nbd.2021.105513_bb0050","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.neuro.2017.06.005","article-title":"The effect of manganese exposure in Atp13a2-deficient mice","volume":"64","author":"Fleming","year":"2018","journal-title":"Neurotoxicology."},{"key":"10.1016\/j.nbd.2021.105513_bb0055","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1212\/WNL.0000000000001315","article-title":"Differential effects of severe vs mild GBA mutations on Parkinson disease","volume":"84","author":"Gan-Or","year":"2015","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105513_bb0060","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1007\/s11910-018-0860-4","article-title":"GBA-associated Parkinson\u2019s disease and other synucleinopathies","volume":"18","author":"Gan-Or","year":"2018","journal-title":"Curr. Neurol. Neurosci. Reports"},{"key":"10.1016\/j.nbd.2021.105513_bb0065","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13024-019-0335-3","article-title":"Microglia affect \u03b1-synuclein cell-to-cell transfer in a mouse model of Parkinson\u2019s disease","volume":"14","author":"George","year":"2019","journal-title":"Mol. Neurodegener."},{"key":"10.1016\/j.nbd.2021.105513_bb0070","doi-asserted-by":"crossref","first-page":"17497","DOI":"10.1074\/jbc.RA120.013428","article-title":"Fibrillar \u03b1-synuclein toxicity depends on functional lysosomes","volume":"295","author":"Guiney","year":"2020","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2021.105513_bb0075","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3389\/fncel.2020.00045","article-title":"Proteostasis of \u03b1-Synuclein and its role in the pathogenesis of Parkinson\u2019s disease","volume":"14","author":"Han","year":"2020","journal-title":"Front. Cell. Neurosci."},{"key":"10.1016\/j.nbd.2021.105513_bb0080","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0162717","article-title":"Alpha-synuclein proteins promote pro-inflammatory cascades in microglia: stronger effects of the A53T mutant","volume":"11","author":"Hoenen","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2021.105513_bb0085","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.tins.2018.09.007","article-title":"Triggers, facilitators, and aggravators: redefining Parkinson\u2019s disease pathogenesis","volume":"42","author":"Johnson","year":"2019","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2021.105513_bb0090","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41598-020-66201-8","article-title":"Deficits in olfactory sensitivity in a mouse model of Parkinson\u2019s disease revealed by plethysmography of odor-evoked sniffing","volume":"10","author":"Johnson","year":"2020","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2021.105513_bb0095","doi-asserted-by":"crossref","first-page":"2665","DOI":"10.1002\/mds.23272","article-title":"Impaired sense of smell and color discrimination in monogenic and idiopathic Parkinson\u2019s disease","volume":"25","author":"Kertelge","year":"2010","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105513_bb0100","doi-asserted-by":"crossref","first-page":"5724","DOI":"10.1523\/JNEUROSCI.0632-14.2015","article-title":"\u03b1-Synuclein-independent histopathological and motor deficits in mice lacking the endolysosomal parkinsonism protein Atp13a2","volume":"35","author":"Kett","year":"2015","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2021.105513_bb0105","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1007\/s40263-019-00620-8","article-title":"Current and emerging treatment strategies for neuronal ceroid lipofuscinoses","volume":"33","author":"Kohlsch\u00fctter","year":"2019","journal-title":"CNS Drugs."},{"key":"10.1016\/j.nbd.2021.105513_bb0110","doi-asserted-by":"crossref","first-page":"1727","DOI":"10.1212\/01.wnl.0000335167.72412.68","article-title":"Novel ATP13A2 variant associated with Parkinson disease in Taiwan and Singapore","volume":"71","author":"Lin","year":"2008","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2021.105513_bb0115","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1111\/bpa.12718","article-title":"The center of olfactory bulb-seeded \u03b1-synucleinopathy is the limbic system and the ensuing pathology is higher in male than in female mice","volume":"29","author":"Mason","year":"2019","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2021.105513_bb0120","doi-asserted-by":"crossref","first-page":"2706","DOI":"10.1093\/brain\/awx221","article-title":"The L444P Gba1 mutation enhances alpha-synuclein induced loss of nigral dopaminergic neurons in mice","volume":"140","author":"Migdalska-Richards","year":"2017","journal-title":"Brain."},{"key":"10.1016\/j.nbd.2021.105513_bb0125","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0238075","article-title":"L444P Gba1 mutation increases formation and spread of \u03b1-synuclein deposits in mice injected with mouse \u03b1-synuclein pre-formed fibrils","volume":"15","author":"Migdalska-Richards","year":"2020","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2021.105513_bb0130","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1002\/mds.28375","article-title":"Brain microglial activation increased in Glucocerebrosidase (GBA) mutation carriers without Parkinson\u2019s disease","volume":"36","author":"Mullin","year":"2021","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105513_bb0135","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1111\/j.1600-0404.1994.tb02645.x","article-title":"Pallido-pyramidal degeneration, supranuclear upgaze paresis and dementia: Kufor-Rakeb syndrome","volume":"89","author":"Najim Al-Din","year":"1994","journal-title":"Acta Neurol. Scand."},{"key":"10.1016\/j.nbd.2021.105513_bb0140","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1002\/humu.21527","article-title":"Pathogenic effects of novel mutations in the P-type ATPase ATP13A2 (PARK9) causing Kufor-Rakeb syndrome, a form of early-onset parkinsonism","volume":"32","author":"Park","year":"2011","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.nbd.2021.105513_bb0145","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1002\/mds.26243","article-title":"The role of ATP13A2 in Parkinson\u2019s disease: clinical phenotypes and molecular mechanisms","volume":"30","author":"Park","year":"2015","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105513_bb0150","first-page":"33","article-title":"Inhibiting the mitochondrial pyruvate carrier does not ameliorate synucleinopathy in the absence of inflammation or metabolic deficits","volume":"1","author":"Peelaerts","year":"2020","journal-title":"Free Neuropathol."},{"key":"10.1016\/j.nbd.2021.105513_bb0155","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1038\/ng1884","article-title":"Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase","volume":"38","author":"Ramirez","year":"2006","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2021.105513_bb0160","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.mcn.2018.05.009","article-title":"Partial loss of ATP13A2 causes selective gliosis independent of robust lipofuscinosis","volume":"92","author":"Rayaprolu","year":"2018","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.nbd.2021.105513_bb0165","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1084\/jem.20160368","article-title":"Widespread transneuronal propagation of \u03b1-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson\u2019s disease","volume":"213","author":"Rey","year":"2016","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.nbd.2021.105513_bb0170","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1007\/s00401-017-1792-9","article-title":"Spread of aggregates after olfactory bulb injection of \u03b1-synuclein fibrils is associated with early neuronal loss and is reduced long term","volume":"135","author":"Rey","year":"2018","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.nbd.2021.105513_bb0175","doi-asserted-by":"crossref","first-page":"364","DOI":"10.3390\/cells8040364","article-title":"GBA, Gaucher disease, and Parkinson\u2019s disease: from genetic to clinic to new therapeutic approaches","volume":"8","author":"Riboldi","year":"2019","journal-title":"Cells."},{"key":"10.1016\/j.nbd.2021.105513_bb0180","doi-asserted-by":"crossref","first-page":"12101","DOI":"10.1073\/pnas.1108197108","article-title":"CNS expression of glucocerebrosidase corrects \u03b1-synuclein pathology and memory in a mouse model of Gaucher-related synucleinopathy","volume":"108","author":"Sardi","year":"2011","journal-title":"Proc. Natl. Acad. Sci."},{"key":"10.1016\/j.nbd.2021.105513_bb0185","doi-asserted-by":"crossref","first-page":"3074","DOI":"10.1016\/j.ajpath.2016.08.006","article-title":"Lysosomal storage of subunit c of mitochondrial ATP synthase in brain-specific Atp13a2-deficient mice","volume":"186","author":"Sato","year":"2016","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.nbd.2021.105513_bb0190","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1002\/mds.22947","article-title":"ATP13A2 mutations (PARK9) cause neurodegeneration with brain iron accumulation","volume":"25","author":"Schneider","year":"2010","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2021.105513_bb0195","doi-asserted-by":"crossref","first-page":"2067","DOI":"10.1093\/hmg\/ddt057","article-title":"Atp13a2-deficient mice exhibit neuronal ceroid lipofuscinosis, limited \u03b1-synuclein accumulation and age-dependent sensorimotor deficits","volume":"22","author":"Schultheis","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2021.105513_bb0200","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41598-018-32217-4","article-title":"Genetic analysis of ATP13A2, PLA2G6 and FBXO7 in a cohort of Chinese patients with early-onset Parkinson\u2019s disease","volume":"8","author":"Shen","year":"2018","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2021.105513_bb0205","doi-asserted-by":"crossref","first-page":"1651","DOI":"10.1056\/NEJMoa0901281","article-title":"Multicenter analysis of glucocerebrosidase mutations in Parkinson\u2019s disease","volume":"361","author":"Sidransky","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.nbd.2021.105513_bb0210","doi-asserted-by":"crossref","first-page":"9617","DOI":"10.1523\/JNEUROSCI.1525-17.2017","article-title":"Glucosylsphingosine promotes \u03b1-synuclein pathology in mutant GBA-associated Parkinson\u2019s disease","volume":"37","author":"Taguchi","year":"2017","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2021.105513_bb0215","doi-asserted-by":"crossref","first-page":"2135","DOI":"10.1038\/nprot.2014.143","article-title":"Addition of exogenous \u03b1-synuclein preformed fibrils to primary neuronal cultures to seed recruitment of endogenous \u03b1-synuclein to Lewy body and Lewy neurite\u2013like aggregates","volume":"9","author":"Volpicelli-Daley","year":"2014","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.nbd.2021.105513_bb0220","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.conb.2017.09.005","article-title":"Endo-lysosomal dysfunction: a converging mechanism in neurodegenerative diseases","volume":"48","author":"Wang","year":"2018","journal-title":"Curr. Opin. Neurobiol."},{"key":"10.1016\/j.nbd.2021.105513_bb0225","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1093\/chemse\/bjn029","article-title":"Sniffing behavior of mice during performance in odor-guided tasks","volume":"33","author":"Wesson","year":"2008","journal-title":"Chem. Senses"},{"key":"10.1016\/j.nbd.2021.105513_bb0230","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13024-017-0233-5","article-title":"\u03b1-Synuclein accumulation and GBA deficiency due to L444P GBA mutation contributes to MPTP-induced parkinsonism","volume":"13","author":"Yun","year":"2018","journal-title":"Mol. Neurodegener."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S096999612100262X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S096999612100262X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:30:32Z","timestamp":1760851832000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S096999612100262X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,11]]},"references-count":47,"alternative-id":["S096999612100262X"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2021.105513","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2021,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Heterozygous GBA D409V and ATP13a2 mutations do not exacerbate pathological \u03b1-synuclein spread in the prodromal preformed fibrils model in young mice","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2021.105513","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"105513"}}