{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T15:41:13Z","timestamp":1783698073174,"version":"3.55.0"},"reference-count":50,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T00:00:00Z","timestamp":1696118400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T00:00:00Z","timestamp":1696118400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T00:00:00Z","timestamp":1694476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004442","name":"Narodowym Centrum Nauki","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004442","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004281","name":"National Science Centre Poland","doi-asserted-by":"publisher","award":["2018\/29\/N\/NZ2\/00802"],"award-info":[{"award-number":["2018\/29\/N\/NZ2\/00802"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Archives of Biochemistry and Biophysics"],"published-print":{"date-parts":[[2023,10]]},"DOI":"10.1016\/j.abb.2023.109754","type":"journal-article","created":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T20:00:13Z","timestamp":1694548813000},"page":"109754","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":4,"special_numbering":"C","title":["Therapeutic potential of lithium chloride and valproic acid against neuronopathic types of mucopolysaccharidoses through induction of the autophagy process"],"prefix":"10.1016","volume":"747","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0820-7204","authenticated-orcid":false,"given":"Lidia","family":"Gaffke","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Firyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estera","family":"Rintz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina","family":"Pierzynowska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewa","family":"Piotrowska","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"Mazur \u2013 Marzec","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grzegorz","family":"W\u0119grzyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib1","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1111\/j.1651-2227.2008.01153.x","article-title":"Correlation between severity of mucopolysaccharidoses and combination of the residual enzyme activity and efficiency of glycosaminoglycan synthesis","volume":"98","author":"Piotrowska","year":"2009","journal-title":"Acta Paediatr."},{"key":"10.1016\/j.abb.2023.109754_bib2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.5582\/irdr.2020.01011","article-title":"A basic understanding of mucopolysaccharidosis: incidence, clinical features, diagnosis, and management","volume":"1","author":"Zhou","year":"2020","journal-title":"Intractable Rare Dis. Res."},{"issue":"3","key":"10.1016\/j.abb.2023.109754_bib3","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1007\/s13353-022-00703-1","article-title":"Misdiagnosis in mucopolysaccharidoses","volume":"63","author":"Wi\u015bniewska","year":"2022","journal-title":"J. Appl. Genet."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib4","doi-asserted-by":"crossref","first-page":"477","DOI":"10.3390\/ijms24010477","article-title":"Mucopolysaccharidoses: cellular consequences of glycosaminoglycans accumulation and potential targets","volume":"24","author":"Leal","year":"2022","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.abb.2023.109754_bib5","article-title":"Non-invasive intravenous administration of AAV9 transducing iduronate sulfatase leads to global metabolic correction and prevention of neurologic deficits in a mouse model of Hunter syndrome","volume":"34","author":"Laoharawee","year":"2023","journal-title":"Mol. Genet. Metab. Rep."},{"key":"10.1016\/j.abb.2023.109754_bib6","first-page":"1","article-title":"An overview of the molecular mechanism of autophagy","volume":"335","author":"Yang","year":"2009","journal-title":"Curr. Top. Microbiol. Immunol."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib7","article-title":"Autophagy in the eye: from physiology to Pathophysology","volume":"2","author":"Liton","year":"2023","journal-title":"Autophagy Rep."},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib8","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1007\/s11011-018-0214-6","article-title":"Autophagy stimulation as a promising approach in treatment of neurodegenerative diseases","volume":"33","author":"Pierzynowska","year":"2018","journal-title":"Metab. Brain Dis."},{"issue":"2","key":"10.1016\/j.abb.2023.109754_bib9","doi-asserted-by":"crossref","first-page":"135","DOI":"10.2217\/fnl-2016-0003","article-title":"Inositol depletion, GSK3 inhibition and bipolar disorder","volume":"11","author":"Yu","year":"2016","journal-title":"Future Neurol."},{"issue":"6","key":"10.1016\/j.abb.2023.109754_bib10","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1021\/cn5000309","article-title":"Neuroprotective effects of lithium: implications for the treatment of Alzheimer's disease and related neurodegenerative disorders","volume":"5","author":"Forlenza","year":"2014","journal-title":"ACS Chem. Neurosci."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib11","doi-asserted-by":"crossref","first-page":"95","DOI":"10.2174\/1874467210902010095","article-title":"Biochemical, molecular and epigenetic mechanisms of valproic acid neuroprotection","volume":"2","author":"Monti","year":"2009","journal-title":"Curr. Mol. Pharmacol."},{"issue":"2","key":"10.1016\/j.abb.2023.109754_bib12","doi-asserted-by":"crossref","first-page":"132","DOI":"10.4161\/auto.2387","article-title":"Inositol and IP3 levels regulate autophagy: biology and therapeutic speculations","volume":"2","author":"Sarkar","year":"2006","journal-title":"Autophagy"},{"key":"10.1016\/j.abb.2023.109754_bib13","doi-asserted-by":"crossref","DOI":"10.1155\/2017\/4629495","article-title":"Multifaceted roles of GSK-3 in cancer and autophagy-related diseases","volume":"2017","author":"Mancinelli","year":"2017","journal-title":"Oxid. Med. Cell. Longev."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib14","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s12017-019-08559-1","article-title":"Mucopolysaccharidosis and autophagy: controversies on the contribution of the process to the pathogenesis and possible therapeutic applications","volume":"22","author":"Pierzynowska","year":"2020","journal-title":"NeuroMolecular Med."},{"key":"10.1016\/j.abb.2023.109754_bib15","doi-asserted-by":"crossref","DOI":"10.1016\/j.ejphar.2020.173534","article-title":"Has resveratrol a potential for mucopolysaccharidosis treatment?","volume":"888","author":"Rintz","year":"2020","journal-title":"Eur. J. Pharmacol."},{"issue":"11","key":"10.1016\/j.abb.2023.109754_bib16","doi-asserted-by":"crossref","first-page":"3875","DOI":"10.1080\/15548627.2021.1961076","article-title":"Protein aggregation and autophagy dysfunction: new lessons from mucopolysaccharidoses","volume":"17","author":"Monaco","year":"2021","journal-title":"Autophagy"},{"key":"10.1016\/j.abb.2023.109754_bib17","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/j.freeradbiomed.2021.12.312","article-title":"An investigation of different intracellular parameters for Inborn Errors of Metabolism: cellular stress, antioxidant response and autophagy","volume":"179","author":"Vardar Acar","year":"2022","journal-title":"Free Radic. Biol. Med."},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib18","doi-asserted-by":"crossref","first-page":"1432","DOI":"10.1016\/j.ymthe.2022.01.037","article-title":"Fluoxetine ameliorates mucopolysaccharidosis type IIIA","volume":"30","author":"Capuozzo","year":"2022","journal-title":"Mol. Ther."},{"issue":"7","key":"10.1016\/j.abb.2023.109754_bib19","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbadis.2022.166399","article-title":"Adipose deficiency and aberrant autophagy in a Drosophila model of MPS VII is corrected by pharmacological stimulators of mTOR","volume":"1868","author":"Basu","year":"2022","journal-title":"Biochim. Biophys. Acta, Mol. Basis Dis."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib20","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1007\/s13311-022-01323-7","article-title":"Activities of (Poly)phenolic antioxidants and other natural autophagy modulators in the treatment of sanfilippo disease: remarkable efficacy of resveratrol in cellular and animal models","volume":"20","author":"Rintz","year":"2023","journal-title":"Neurotherapeutics"},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib21","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1590\/S1415-47572012000600006","article-title":"Mucopolysacccharidoses: from understanding to treatment, a century of discoveries","volume":"35","author":"Giugliani","year":"2012","journal-title":"Genet. Mol. Biol."},{"issue":"Suppl 1","key":"10.1016\/j.abb.2023.109754_bib22","doi-asserted-by":"crossref","first-page":"S10","DOI":"10.1016\/j.pedneo.2022.10.001","article-title":"Current and new therapies for mucopolysaccharidoses","volume":"64","author":"Penon-Portmann","year":"2023","journal-title":"Pediatr. Neonatol."},{"issue":"Suppl 5","key":"10.1016\/j.abb.2023.109754_bib23","doi-asserted-by":"crossref","first-page":"v4","DOI":"10.1093\/rheumatology\/ker394","article-title":"Overview of the mucopolysaccharidoses","volume":"50","author":"Muenzer","year":"2011","journal-title":"Rheumatology"},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib24","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1081\/CLT-100105159","article-title":"The effects of lithium, valproic acid, and carbamazepine during pregnancy and lactation","volume":"39","author":"Iqbal","year":"2001","journal-title":"J. Toxicol. Clin. Toxicol."},{"issue":"5","key":"10.1016\/j.abb.2023.109754_bib25","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1111\/j.1471-4159.1985.tb08793.x","article-title":"Blood-brain barrier transport of valproic acid","volume":"44","author":"Cornford","year":"1985","journal-title":"J. Neurochem."},{"issue":"5511","key":"10.1016\/j.abb.2023.109754_bib26","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1038\/255723a0","article-title":"Sodium transport and lithium movements across the insect blood-brain barrier","volume":"255","author":"Schofield","year":"1975","journal-title":"Nature"},{"issue":"6938","key":"10.1016\/j.abb.2023.109754_bib27","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1038\/nature01640","article-title":"GSK-3alpha regulates production of Alzheimer's disease amyloid-beta peptides","volume":"423","author":"Phiel","year":"2003","journal-title":"Nature"},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib28","doi-asserted-by":"crossref","first-page":"739","DOI":"10.3233\/JAD-2011-101875","article-title":"Long-term treatment with lithium alleviates memory deficits and reduces amyloid-\u03b2 production in an aged Alzheimer's disease transgenic mouse model","volume":"24","author":"Zhang","year":"2011","journal-title":"J. Alzheimers Dis."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib29","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1007\/s12035-014-8751-4","article-title":"Valproic acid alleviates memory deficits and attenuates amyloid-\u03b2 deposition in transgenic mouse model of Alzheimer's disease","volume":"51","author":"Xuan","year":"2015","journal-title":"Mol. Neurobiol."},{"issue":"6","key":"10.1016\/j.abb.2023.109754_bib30","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1007\/s00210-017-1357-4","article-title":"Protective effect of valproic acid in streptozotocin-induced sporadic Alzheimer's disease mouse model: possible involvement of the cholinergic system","volume":"390","author":"Sorial","year":"2017","journal-title":"Naunyn-Schmiedeberg\u2019s Arch. Pharmacol."},{"key":"10.1016\/j.abb.2023.109754_bib31","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1002\/gps.1964","article-title":"A feasibility and tolerability study of lithium in Alzheimer's disease","volume":"7","author":"Macdonald","year":"2008","journal-title":"Int. J. Geriatr. Psychiatr."},{"key":"10.1016\/j.abb.2023.109754_bib32","doi-asserted-by":"crossref","first-page":"553","DOI":"10.2174\/156720505774932205","article-title":"A randomized, double-blind, placebo-controlled pilot trial of safety and tolerability of two doses of divalproex sodium in outpatients with probable Alzheimer's disease","volume":"5","author":"Profenno","year":"2005","journal-title":"Curr. Alzheimer Res."},{"issue":"4","key":"10.1016\/j.abb.2023.109754_bib33","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/S0361-9230(03)00141-2","article-title":"Chronic lithium chloride treatment has variable effects on motor behaviour and survival of mice transgenic for the Huntington's disease mutation","volume":"61","author":"Wood","year":"2003","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.abb.2023.109754_bib34","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/j.pbb.2009.08.001","article-title":"Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease","volume":"1","author":"Z\u00e1dori","year":"2009","journal-title":"Pharmacol. Biochem. Behav."},{"key":"10.1016\/j.abb.2023.109754_bib35","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.brainres.2014.10.032","article-title":"Lithium prevents parkinsonian behavioral and striatal phenotypes in an aged parkin mutant transgenic mouse model","volume":"1591","author":"Lieu","year":"2014","journal-title":"Brain Res."},{"key":"10.1016\/j.abb.2023.109754_bib36","article-title":"Valproic acid neuroprotection in the 6-OHDA model of Parkinson's disease is possibly related to its anti-inflammatory and HDAC inhibitory properties","volume":"2015","author":"Ximenes","year":"2015","journal-title":"J. Neurodegener. Dis."},{"issue":"3","key":"10.1016\/j.abb.2023.109754_bib37","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1017\/S0317167100023970","article-title":"Treatment of Parkinson's disease with sodium valproate: clinical, pharmacological, and biochemical observations","volume":"6","author":"Nutt","year":"1979","journal-title":"Can. J. Neurol. Sci."},{"issue":"21","key":"10.1016\/j.abb.2023.109754_bib38","doi-asserted-by":"crossref","first-page":"5535","DOI":"10.1523\/JNEUROSCI.1139-07.2007","article-title":"Sodium valproate exerts neuroprotective effects in vivo through CREB-binding protein-dependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model","volume":"27","author":"Rouaux","year":"2007","journal-title":"J. Neurosci."},{"issue":"6","key":"10.1016\/j.abb.2023.109754_bib39","doi-asserted-by":"crossref","first-page":"2052","DOI":"10.1073\/pnas.0708022105","article-title":"Lithium delays progression of amyotrophic lateral sclerosis","volume":"105","author":"Fornai","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.abb.2023.109754_bib40","article-title":"A phase 2, double-blind, placebo-controlled trial of a valproate\/lithium combination in ALS patients","volume":"29","author":"Boll","year":"2022","journal-title":"Neurologia"},{"key":"10.1016\/j.abb.2023.109754_bib41","doi-asserted-by":"crossref","DOI":"10.1016\/j.bone.2021.116237","article-title":"Effects of lithium administration on vertebral bone disease in mucopolysaccharidosis I dogs","volume":"154","author":"Lau","year":"2022","journal-title":"Bone"},{"issue":"7","key":"10.1016\/j.abb.2023.109754_bib42","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1038\/sj.ejhg.5201623","article-title":"Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses","volume":"14","author":"Piotrowska","year":"2006","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1016\/j.abb.2023.109754_bib43","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1007\/s12017-018-8482-1","article-title":"Correction of Huntington's disease phenotype by genistein-induced autophagy in the cellular model","volume":"1","author":"Pierzynowska","year":"2018","journal-title":"NeuroMolecular Med."},{"key":"10.1016\/j.abb.2023.109754_bib44","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.neuropharm.2019.01.030","article-title":"Autophagy-dependent mechanism of genistein-mediated elimination of behavioral and biochemical defects in the rat model of sporadic Alzheimer's disease","volume":"148","author":"Pierzynowska","year":"2019","journal-title":"Neuropharmacology"},{"issue":"12","key":"10.1016\/j.abb.2023.109754_bib45","doi-asserted-by":"crossref","first-page":"278","DOI":"10.3390\/cells7120278","article-title":"Autophagy: an essential degradation program for cellular homeostasis and life","volume":"7","author":"Chun","year":"2018","journal-title":"Cells"},{"key":"10.1016\/j.abb.2023.109754_bib46","first-page":"19","article-title":"Newborn screening and diagnosis of mucopolysaccharidoses: application of tandem mass spectrometry","volume":"24","author":"Tomatsu","year":"2014","journal-title":"Nihon Masu Sukuriningu Gakkai Shi"},{"issue":"15","key":"10.1016\/j.abb.2023.109754_bib47","doi-asserted-by":"crossref","first-page":"5449","DOI":"10.3390\/ijms21155449","article-title":"High-throughput liquid chromatography-tandem mass spectrometry quantification of glycosaminoglycans as biomarkers of mucopolysaccharidosis II","volume":"21","author":"Wang","year":"2020","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.abb.2023.109754_bib48","series-title":"StatPearls. Treasure Island (FL)","article-title":"Valproate toxicity","author":"Patel","year":"2022"},{"key":"10.1016\/j.abb.2023.109754_bib49","first-page":"70","article-title":"The toxic effects, excretion and distribution of lithium chloride","volume":"100","author":"Radomsky","year":"1950","journal-title":"J. Pharmacol. Exp. Therapeut."},{"issue":"1","key":"10.1016\/j.abb.2023.109754_bib50","doi-asserted-by":"crossref","first-page":"134","DOI":"10.3390\/metabo13010134","article-title":"Therapeutic and toxic effects of valproic acid metabolites","volume":"13","author":"Shnayder","year":"2023","journal-title":"Metabolites"}],"container-title":["Archives of Biochemistry and Biophysics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0003986123002539?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0003986123002539?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,13]],"date-time":"2025-09-13T06:26:22Z","timestamp":1757744782000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0003986123002539"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10]]},"references-count":50,"alternative-id":["S0003986123002539"],"URL":"https:\/\/doi.org\/10.1016\/j.abb.2023.109754","relation":{},"ISSN":["0003-9861"],"issn-type":[{"value":"0003-9861","type":"print"}],"subject":[],"published":{"date-parts":[[2023,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Therapeutic potential of lithium chloride and valproic acid against neuronopathic types of mucopolysaccharidoses through induction of the autophagy process","name":"articletitle","label":"Article Title"},{"value":"Archives of Biochemistry and Biophysics","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.abb.2023.109754","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"109754"}}