{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T04:54:10Z","timestamp":1790830450223,"version":"4.1.0"},"reference-count":102,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,1,1]],"date-time":"2017-01-01T00:00:00Z","timestamp":1483228800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"name":"TEVA Pharmaceuticals","award":["SRA #109415"],"award-info":[{"award-number":["SRA #109415"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS074376"],"award-info":[{"award-number":["R01NS074376"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01NS056224"],"award-info":[{"award-number":["R01NS056224"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["F32NS093786"],"award-info":[{"award-number":["F32NS093786"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2017,1]]},"DOI":"10.1016\/j.nbd.2016.10.006","type":"journal-article","created":{"date-parts":[[2016,11,3]],"date-time":"2016-11-03T05:00:59Z","timestamp":1478149259000},"page":"46-59","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":131,"special_numbering":"PA","title":["The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease"],"prefix":"10.1016","volume":"97","author":[{"given":"Daniel","family":"Ryskamp","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Geva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Kusko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"Grossman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Hayden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7006-6951","authenticated-orcid":false,"given":"Ilya","family":"Bezprozvanny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2016.10.006_bb0005","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.ceca.2011.05.008","article-title":"Investigation of the role of sigma1-receptors in inositol 1,4,5-trisphosphate dependent calcium signaling in hepatocytes","volume":"50","author":"Abou-Lovergne","year":"2011","journal-title":"Cell Calcium"},{"key":"10.1016\/j.nbd.2016.10.006_bb0010","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1002\/ana.22534","article-title":"A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis","volume":"70","author":"Al-Saif","year":"2011","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0015","doi-asserted-by":"crossref","first-page":"8710","DOI":"10.1523\/JNEUROSCI.20-23-08710.2000","article-title":"Dendritic and axonal targeting of type 5 metabotropic glutamate receptor is regulated by homer1 proteins and neuronal excitation","volume":"20","author":"Ango","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0020","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1038\/35082096","article-title":"Agonist-independent activation of metabotropic glutamate receptors by the intracellular protein Homer","volume":"411","author":"Ango","year":"2001","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2016.10.006_bb0025","doi-asserted-by":"crossref","first-page":"569","DOI":"10.3233\/JAD-2011-101794","article-title":"Anti-amnesic and neuroprotective actions of the sigma-1 receptor agonist (\u2212)-MR22 in rats with selective cholinergic lesion and amyloid infusion","volume":"24","author":"Antonini","year":"2011","journal-title":"J. Alzheimers Dis."},{"key":"10.1016\/j.nbd.2016.10.006_bb0030","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1111\/j.1471-4159.2009.06000.x","article-title":"Anti-amnesic properties of (\u00b1)-PPCC, a novel sigma receptor ligand, on cognitive dysfunction induced by selective cholinergic lesion in rats","volume":"109","author":"Antonini","year":"2009","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2016.10.006_bb0035","doi-asserted-by":"crossref","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat. Rev. Dis. Primers."},{"key":"10.1016\/j.nbd.2016.10.006_bb0040","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1038\/ejhg.2012.135","article-title":"Genetic analysis of SIGMAR1 as a cause of familial ALS with dementia","volume":"21","author":"Belzil","year":"2013","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1016\/j.nbd.2016.10.006_bb0045","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1093\/brain\/awv008","article-title":"Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration","volume":"138","author":"Bernard-Marissal","year":"2015","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2016.10.006_bb0050","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/1750-1326-8-23","article-title":"The synaptic maintenance problem: membrane recycling, Ca2+ homeostasis and late onset degeneration","volume":"8","author":"Bezprozvanny","year":"2013","journal-title":"Mol. Neurodegener."},{"key":"10.1016\/j.nbd.2016.10.006_bb0055","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/BJ20150934","article-title":"Cocaine inhibits store-operated Ca2+ entry in brain microvascular endothelial cells: critical role for sigma-1 receptors","volume":"473","author":"Brailoiu","year":"2016","journal-title":"Biochem. J."},{"key":"10.1016\/j.nbd.2016.10.006_bb0060","doi-asserted-by":"crossref","first-page":"2993","DOI":"10.1021\/bi401575g","article-title":"The sigma enigma: in vitro\/in silico site-directed mutagenesis studies unveil sigma1 receptor ligand binding","volume":"53","author":"Brune","year":"2014","journal-title":"Biochemistry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0065","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1042\/bj3410795","article-title":"Co-expression of metabotropic glutamate receptor type 1\u03b1 with Homer-1a\/Vesl-1S increases the cell surface expression of the receptor","volume":"341","author":"Ciruela","year":"1999","journal-title":"Biochem. J."},{"key":"10.1016\/j.nbd.2016.10.006_bb0070","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.ejphar.2009.11.025","article-title":"The dopaminergic stabilizers pridopidine (ACR16) and (\u2212)-OSU6162 display dopamine D(2) receptor antagonism and fast receptor dissociation properties","volume":"628","author":"Dyhring","year":"2010","journal-title":"Eur. J. Pharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0075","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1097\/WNF.0b013e31821c31d8","article-title":"Regional cerebral glucose metabolism after pridopidine (ACR16) treatment in patients with Huntington disease","volume":"34","author":"Esmaeilzadeh","year":"2011","journal-title":"Clin. Neuropharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0080","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1016\/j.neulet.2012.04.046","article-title":"Association between a variant of the sigma-1 receptor gene and Alzheimer's disease","volume":"517","author":"Feh\u00e9r","year":"2012","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.nbd.2016.10.006_bb0085","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1159\/000440864","article-title":"AF710B, a novel M1\/\u03c31 agonist with therapeutic efficacy in animal models of Alzheimer's disease","volume":"16","author":"Fisher","year":"2016","journal-title":"Neurodegener. Dis."},{"key":"10.1016\/j.nbd.2016.10.006_bb0090","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1136\/jnnp.66.1.52","article-title":"Differences in duration of Huntington's disease based on age at onset","volume":"66","author":"Foroud","year":"1999","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0095","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1093\/brain\/awu107","article-title":"Pharmacological stimulation of sigma-1 receptors has neurorestorative effects in experimental parkinsonism","volume":"137","author":"Francardo","year":"2014","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2016.10.006_bb0100","doi-asserted-by":"crossref","DOI":"10.1093\/hmg\/ddw238","article-title":"Pridopidine activates neuroprotective pathways impaired in Huntington disease","author":"Geva","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2016.10.006_bb0105","doi-asserted-by":"crossref","first-page":"7749","DOI":"10.1167\/iovs.11-8169","article-title":"Late-onset inner retinal dysfunction in mice lacking sigma receptor 1 (\u03c3R1)","volume":"52","author":"Ha","year":"2011","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0110","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1124\/jpet.103.051284","article-title":"\u03c3-1 receptors (\u03c31 binding sites) form raft-like microdomains and target lipid droplets on the endoplasmic reticulum: roles in endoplasmic reticulum lipid compartmentalization and export","volume":"306","author":"Hayashi","year":"2003","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"10.1016\/j.nbd.2016.10.006_bb0115","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1016\/j.cell.2007.08.036","article-title":"Sigma-1 receptor chaperones at the ER-mitochondrion interface regulate Ca(2+) signaling and cell survival","volume":"131","author":"Hayashi","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2016.10.006_bb0120","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1002\/hup.1106","article-title":"Cognition and depression: the effects of fluvoxamine, a sigma-1 receptor agonist, reconsidered","volume":"25","author":"Hindmarch","year":"2010","journal-title":"Hum. Psychopharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0125","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1016\/0006-8993(95)01393-8","article-title":"The presence of calbindin in rat cortical neurons protects in vitro from oxydative stress","volume":"707","author":"Hugon","year":"1996","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2016.10.006_bb0130","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1002\/mds.25362","article-title":"A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease","volume":"28","author":"Huntington Study Group HART Investigators","year":"2013","journal-title":"Mov. Disord."},{"key":"10.1016\/j.nbd.2016.10.006_bb0135","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2013.170","article-title":"Sigma-1 receptor agonist PRE084 is protective against mutant huntingtin-induced cell degeneration: involvement of calpastatin and the NF-\u03baB pathway","volume":"4","author":"Hyrskyluoto","year":"2013","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.nbd.2016.10.006_bb0140","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1002\/iub.1162","article-title":"Homer proteins in Ca2+ entry","volume":"65","author":"Jardin","year":"2013","journal-title":"IUBMB Life"},{"key":"10.1016\/j.nbd.2016.10.006_bb0145","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1038\/nprot.2006.86","article-title":"High Ca2+-phosphate transfection efficiency in low-density neuronal cultures","volume":"1","author":"Jiang","year":"2006","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.nbd.2016.10.006_bb0150","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/0006-8993(90)90866-A","article-title":"Calbindin D28K as a marker for the degeneration of the striatonigral pathway in Huntington's disease","volume":"525","author":"Kiyama","year":"1990","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2016.10.006_bb0155","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1016\/j.tins.2012.09.007","article-title":"The sigma-1 receptor: roles in neuronal plasticity and disease","volume":"35","author":"Kourrich","year":"2012","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0160","doi-asserted-by":"crossref","first-page":"2188","DOI":"10.1046\/j.1460-9568.2003.02950.x","article-title":"Generation and phenotypic analysis of sigma receptor type I (\u03c31) knockout mice","volume":"18","author":"Langa","year":"2003","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0165","article-title":"Inhibition of store-operated calcium entry attenuates MPP(+)-induced oxidative stress via preservation of mitochondrial function in PC12 cells: involvement of Homer1a","volume":"8","author":"Li","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2016.10.006_bb0170","doi-asserted-by":"crossref","first-page":"2430","DOI":"10.1212\/WNL.0000000000001680","article-title":"A SIGMAR1 splice-site mutation causes distal hereditary motor neuropathy","volume":"84","author":"Li","year":"2015","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2016.10.006_bb0175","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1097\/WNF.0b013e3181ebb285","article-title":"Efficacy and safety of the dopaminergic stabilizer pridopidine (ACR16) in patients with Huntington's disease","volume":"33","author":"Lundin","year":"2010","journal-title":"Clin. Neuropharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0180","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2014.116","article-title":"Postsynaptic scaffold protein Homer 1a protects against traumatic brain injury via regulating group I metabotropic glutamate receptors","volume":"5","author":"Luo","year":"2014","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.nbd.2016.10.006_bb0185","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1002\/ana.22274","article-title":"Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease","volume":"68","author":"Luty","year":"2010","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0190","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","author":"MacDonald","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2016.10.006_bb0195","doi-asserted-by":"crossref","first-page":"2741","DOI":"10.1523\/JNEUROSCI.4360-04.2005","article-title":"The scaffold protein Homer1b\/c links metabotropic glutamate receptor 5 to extracellular signal-regulated protein kinase cascades in neurons","volume":"25","author":"Mao","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0200","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1097\/00001756-200508010-00018","article-title":"Neuroprotective effects of sigma-1 receptor agonists against beta-amyloid-induced toxicity","volume":"16","author":"Marrazzo","year":"2005","journal-title":"Neuroreport"},{"key":"10.1016\/j.nbd.2016.10.006_bb0205","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1159\/000101990","article-title":"Sigma receptor type 1 gene variation in a group of Polish patients with Alzheimer's disease and mild cognitive impairment","volume":"23","author":"Maruszak","year":"2007","journal-title":"Dement. Geriatr. Cogn. Disord."},{"key":"10.1016\/j.nbd.2016.10.006_bb0210","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.pharmthera.2009.07.001","article-title":"The pharmacology of sigma-1 receptors","volume":"124","author":"Maurice","year":"2009","journal-title":"Pharmacol. Ther."},{"key":"10.1016\/j.nbd.2016.10.006_bb0215","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/j.pbb.2006.07.009","article-title":"Neuro (active) steroids actions at the neuromodulatory sigma 1 (\u03c3 1) receptor: biochemical and physiological evidences, consequences in neuroprotection","volume":"84","author":"Maurice","year":"2006","journal-title":"Pharmacol. Biochem. Behav."},{"key":"10.1016\/j.nbd.2016.10.006_bb0220","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.neuroscience.2010.02.022","article-title":"The sigma-1 receptor is enriched in postsynaptic sites of C-terminals in mouse motoneurons. An anatomical and behavioral study","volume":"167","author":"Mavlyutov","year":"2010","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2016.10.006_bb0225","doi-asserted-by":"crossref","first-page":"998","DOI":"10.1038\/sj.bjp.0706927","article-title":"The anti-amnesic and neuroprotective effects of donepezil against amyloid beta25-35 peptide-induced toxicity in mice involve an interaction with the sigma1 receptor","volume":"149","author":"Meunier","year":"2006","journal-title":"Br. J. Pharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0230","doi-asserted-by":"crossref","first-page":"735","DOI":"10.2217\/fnl.10.41","article-title":"Corticostriatal circuit dysfunction in Huntington's disease: intersection of glutamate, dopamine, and calcium","volume":"5","author":"Miller","year":"2010","journal-title":"Future Neurol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0235","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1113\/jphysiol.2007.142448","article-title":"Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load","volume":"585","author":"Milnerwood","year":"2007","journal-title":"J. Physiol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0240","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.tins.2010.08.002","article-title":"Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease","volume":"33","author":"Milnerwood","year":"2010","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0245","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1007\/s12149-007-0094-z","article-title":"Low density of sigma1 receptors in early Alzheimer's disease","volume":"22","author":"Mishina","year":"2008","journal-title":"Ann. Nucl. Med."},{"key":"10.1016\/j.nbd.2016.10.006_bb0250","doi-asserted-by":"crossref","first-page":"12997","DOI":"10.1523\/JNEUROSCI.5284-12.2013","article-title":"Dendritic spine instability leads to progressive neocortical spine loss in a mouse model of Huntington's disease","volume":"33","author":"Murmu","year":"2013","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0255","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1212\/WNL.38.3.341","article-title":"Clinical and neuropathologic assessment of severity in Huntington's disease","volume":"38","author":"Myers","year":"1988","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2016.10.006_bb0260","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jphs.2014.12.005","article-title":"Role of sigma-1 receptors in neurodegenerative diseases","volume":"127","author":"Nguyen","year":"2015","journal-title":"J. Pharmacol. Sci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0265","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/j.pnpbp.2004.05.004","article-title":"The dopaminergic stabiliser ACR16 counteracts the behavioural primitivization induced by the NMDA receptor antagonist MK-801 in mice: implications for cognition","volume":"28","author":"Nilsson","year":"2004","journal-title":"Prog. Neuro-Psychopharmacol. Biol. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0270","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.febslet.2015.01.033","article-title":"Solution NMR studies reveal the location of the second transmembrane domain of the human sigma-1 receptor","volume":"589","author":"Ortega-Roldan","year":"2015","journal-title":"FEBS Lett."},{"key":"10.1016\/j.nbd.2016.10.006_bb0275","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1136\/jnnp.2009.171926","article-title":"Abnormal motor cortex plasticity in premanifest and very early manifest Huntington disease","volume":"81","author":"Orth","year":"2010","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0280","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.ejphar.2012.01.030","article-title":"The sigma-1 receptor protects against cellular oxidative stress and activates antioxidant response elements","volume":"682","author":"Pal","year":"2012","journal-title":"Eur. J. Pharmacol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0285","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1016\/j.cell.2014.09.014","article-title":"CRISPR-Cas9 knockin mice for genome editing and cancer modeling","volume":"159","author":"Platt","year":"2014","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2016.10.006_bb0290","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13024-015-0034-7","article-title":"STIM2 protects hippocampal mushroom spines from amyloid synaptotoxicity","volume":"10","author":"Popugaeva","year":"2015","journal-title":"Mol. Neurodegener."},{"key":"10.1016\/j.nbd.2016.10.006_bb0295","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2016.10.006_bb0300","doi-asserted-by":"crossref","first-page":"25953","DOI":"10.1074\/jbc.274.36.25953","article-title":"Homer 1b regulates the trafficking of group I metabotropic glutamate receptors","volume":"274","author":"Roche","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2016.10.006_bb0305","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0001997","article-title":"Automated three-dimensional detection and shape classification of dendritic spines from fluorescence microscopy images","volume":"3","author":"Rodriguez","year":"2008","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2016.10.006_bb0310","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1007\/s00702-008-0038-3","article-title":"Effects of (\u2212)-OSU6162 and ACR16 on motor activity in rats, indicating a unique mechanism of dopaminergic stabilization","volume":"115","author":"Rung","year":"2008","journal-title":"J. Neural Transm."},{"key":"10.1016\/j.nbd.2016.10.006_bb0315","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1093\/brain\/awq367","article-title":"The sigma-1 receptor enhances brain plasticity and functional recovery after experimental stroke","volume":"134","author":"Ruscher","year":"2011","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2016.10.006_bb0320","series-title":"Neurodegenerative Diseases: Unifying Principles","first-page":"27","article-title":"Calcium hypothesis of neurodegeneration: the case of Alzheimer's disease and Huntington's disease.","author":"Ryskamp","year":"2016"},{"key":"10.1016\/j.nbd.2016.10.006_bb0325","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1038\/mp.2012.3","article-title":"The dopamine stabilizers ACR16 and (\u2212)-OSU6162 display nanomolar affinities at the sigma-1 receptor","volume":"18","author":"Sahlholm","year":"2013","journal-title":"Mol. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0330","doi-asserted-by":"crossref","first-page":"3443","DOI":"10.1007\/s00213-015-3997-8","article-title":"Pridopidine selectively occupies sigma-1 rather than dopamine D2 receptors at behaviorally active doses","volume":"232","author":"Sahlholm","year":"2015","journal-title":"Psychopharmacology"},{"key":"10.1016\/j.nbd.2016.10.006_bb0335","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1038\/nmeth.3047","article-title":"Improved vectors and genome-wide libraries for CRISPR screening","volume":"11","author":"Sanjana","year":"2014","journal-title":"Nat. Methods"},{"key":"10.1016\/j.nbd.2016.10.006_bb0340","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.brainres.2007.08.068","article-title":"A prototypical sigma-1 receptor antagonist protects against brain ischemia","volume":"1181","author":"Schetz","year":"2007","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2016.10.006_bb0345","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1016\/j.biopsych.2008.12.026","article-title":"Abnormal motor cortex excitability in preclinical and very early Huntington's disease","volume":"65","author":"Schippling","year":"2009","journal-title":"Biol. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0350","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1038\/nature17391","article-title":"Crystal structure of the human sigma1 receptor","volume":"532","author":"Schmidt","year":"2016","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2016.10.006_bb0355","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1186\/gb-2007-8-2-206","article-title":"The Homer family proteins","volume":"8","author":"Shiraishi-Yamaguchi","year":"2007","journal-title":"Genome Biol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0360","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1212\/WNL.29.1.1","article-title":"Huntington disease clinical care and evaluation","volume":"29","author":"Shoulson","year":"1979","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2016.10.006_bb0365","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2016.10.006_bb0370","doi-asserted-by":"crossref","first-page":"4154","DOI":"10.1167\/iovs.08-1824","article-title":"In vivo protection against retinal neurodegeneration by sigma receptor 1 ligand (+)-pentazocine","volume":"49","author":"Smith","year":"2008","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0375","doi-asserted-by":"crossref","first-page":"2540","DOI":"10.1111\/jcmm.12604","article-title":"Pridopidine, a dopamine stabilizer, improves motor performance and shows neuroprotective effects in Huntington disease R6\/2 mouse model","volume":"11","author":"Squitieri","year":"2015","journal-title":"J. Cell. Mol. Med."},{"key":"10.1016\/j.nbd.2016.10.006_bb0380","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1083\/jcb.201506022","article-title":"Sigma1 receptors inhibit store-operated Ca2+ entry by attenuating coupling of STIM1 to Orai1","volume":"213","author":"Srivats","year":"2016","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0385","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/j.tips.2010.08.007","article-title":"The sigma-1 receptor chaperone as an inter-organelle signaling modulator","volume":"31","author":"Su","year":"2010","journal-title":"Trends Pharmacol. Sci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0390","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.neuron.2014.02.019","article-title":"Reduced synaptic STIM2 expression and impaired store-operated calcium entry cause destabilization of mature spines in mutant presenilin mice","volume":"82","author":"Sun","year":"2014","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2016.10.006_bb0395","doi-asserted-by":"crossref","first-page":"13801","DOI":"10.1073\/pnas.96.24.13801","article-title":"Involvement of unique leucine-zipper motif of PSD-Zip45 (Homer 1c\/vesl-1L) in group 1 metabotropic glutamate receptor clustering","volume":"96","author":"Tadokoro","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.10.006_bb0400","doi-asserted-by":"crossref","first-page":"S208","DOI":"10.1055\/s-2004-832679","article-title":"A perspective on the new mechanism of antidepressants: neuritogenesis through sigma-1 receptors","volume":"37","author":"Takebayashi","year":"2004","journal-title":"Pharmacopsychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0415","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/S0896-6273(03)00366-0","article-title":"Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1","volume":"39","author":"Tang","year":"2003","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2016.10.006_bb0410","doi-asserted-by":"crossref","first-page":"2602","DOI":"10.1073\/pnas.0409402102","article-title":"Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease","volume":"102","author":"Tang","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.10.006_bb0405","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1523\/JNEUROSCI.4411-08.2009","article-title":"Neuroprotective effects of inositol 1,4,5-trisphosphate receptor C-terminal fragment in a Huntington's disease mouse model","volume":"29","author":"Tang","year":"2009","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0425","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1054\/ceca.1999.0090","article-title":"Expression of inositol trisphosphate receptors","volume":"26","author":"Taylor","year":"1999","journal-title":"Cell Calcium"},{"key":"10.1016\/j.nbd.2016.10.006_bb0420","doi-asserted-by":"crossref","first-page":"22468","DOI":"10.1073\/pnas.0909089106","article-title":"Sigma-1 receptors regulate hippocampal dendritic spine formation via a free radical-sensitive mechanism involving Rac1\u00b7GTP pathway","volume":"106","author":"Tsai","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.10.006_bb0430","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1002\/syn.20984","article-title":"Insights into the sigma-1 receptor chaperone's cellular functions: a microarray report","volume":"66","author":"Tsai","year":"2012","journal-title":"Synapse"},{"key":"10.1016\/j.nbd.2016.10.006_bb0435","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1073\/pnas.1422001112","article-title":"Sigma-1 receptor regulates Tau phosphorylation and axon extension by shaping p35 turnover via myristic acid","volume":"112","author":"Tsai","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.10.006_bb0440","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1016\/S0896-6273(00)80589-9","article-title":"Homer binds a novel proline-rich motif and links group 1 metabotropic glutamate receptors with IP3 receptors","volume":"21","author":"Tu","year":"1998","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2016.10.006_bb0445","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1097\/00019442-200512000-00005","article-title":"A variant of the sigma receptor type-1 gene is a protective factor for Alzheimer disease","volume":"13","author":"Uchida","year":"2005","journal-title":"Am. J. Geriatr. Psychiatry"},{"key":"10.1016\/j.nbd.2016.10.006_bb0450","doi-asserted-by":"crossref","first-page":"1552","DOI":"10.1038\/npp.2008.212","article-title":"Antiamnesic and neuroprotective effects of the aminotetrahydrofuran derivative ANAVEX1-41 against amyloid beta(25-35)-induced toxicity in mice","volume":"34","author":"Villard","year":"2009","journal-title":"Neuropsychopharmacology"},{"key":"10.1016\/j.nbd.2016.10.006_bb0455","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","article-title":"Huntington disease","volume":"57","author":"Vonsattel","year":"1998","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0460","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.freeradbiomed.2015.04.009","article-title":"Sigma 1 receptor regulates the oxidative stress response in primary retinal Muller glial cells via NRF2 signaling and system xc(\u2212), the Na(+)-independent glutamate-cystine exchanger","volume":"86","author":"Wang","year":"2015","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.nbd.2016.10.006_bb0465","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2015.216","article-title":"Scaffolding protein Homer1a protects against NMDA-induced neuronal injury","volume":"6","author":"Wang","year":"2015","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.nbd.2016.10.006_bb0470","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1016\/j.ceca.2007.05.007","article-title":"Homer proteins in Ca2+ signaling by excitable and non-excitable cells","volume":"42","author":"Worley","year":"2007","journal-title":"Cell Calcium"},{"key":"10.1016\/j.nbd.2016.10.006_bb0475","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1523\/JNEUROSCI.1038-15.2016","article-title":"Enhanced store-operated calcium entry leads to striatal synaptic loss in a Huntington's disease mouse model","volume":"36","author":"Wu","year":"2016","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.10.006_bb0480","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/j.chembiol.2011.04.012","article-title":"Neuronal store-operated calcium entry pathway as a novel therapeutic target for Huntington's disease treatment","volume":"18","author":"Wu","year":"2011","journal-title":"Chem. Biol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0485","doi-asserted-by":"crossref","first-page":"28198","DOI":"10.1074\/jbc.M802099200","article-title":"Role of sigma-1 receptor C-terminal segment in inositol 1,4,5-trisphosphate receptor activation: constitutive enhancement of calcium signaling in MCF-7 tumor cells","volume":"283","author":"Wu","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2016.10.006_bb0490","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/0006-8993(90)91236-A","article-title":"Relative sparing in Parkinson's disease of substantia nigra dopamine neurons containing calbindin-D28K","volume":"526","author":"Yamada","year":"1990","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2016.10.006_bb0495","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1016\/S1474-4422(11)70233-2","article-title":"Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial","volume":"10","author":"de Yebenes","year":"2011","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.nbd.2016.10.006_bb0500","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1161\/01.STR.32.4.1028","article-title":"Calbindin d28k overexpression protects striatal neurons from transient focal cerebral ischemia","volume":"32","author":"Yenari","year":"2001","journal-title":"Stroke"},{"key":"10.1016\/j.nbd.2016.10.006_bb0505","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/j.neulet.2013.03.030","article-title":"SKF-96365 attenuates toxin-induced neuronal injury through opposite regulatory effects on Homer1a and Homer1b\/c in cultured rat mesencephalic cells","volume":"543","author":"Zeng","year":"2013","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.nbd.2016.10.006_bb0510","doi-asserted-by":"crossref","first-page":"13275","DOI":"10.1523\/JNEUROSCI.1034-15.2015","article-title":"Neuronal store-operated calcium entry and mushroom spine loss in amyloid precursor protein knock-in mouse model of Alzheimer's disease","volume":"35","author":"Zhang","year":"2015","journal-title":"J. Neurosci."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996116302479?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996116302479?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,9,15]],"date-time":"2019-09-15T03:21:56Z","timestamp":1568517716000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996116302479"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1]]},"references-count":102,"alternative-id":["S0969996116302479"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2016.10.006","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2017,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2016.10.006","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}