{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T00:34:37Z","timestamp":1783384477016,"version":"3.54.6"},"publisher-location":"New York, NY","reference-count":22,"publisher":"Springer US","isbn-type":[{"value":"9781071648438","type":"print"},{"value":"9781071648445","type":"electronic"}],"license":[{"start":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T00:00:00Z","timestamp":1760400000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T00:00:00Z","timestamp":1760400000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026]]},"DOI":"10.1007\/978-1-0716-4844-5_15","type":"book-chapter","created":{"date-parts":[[2025,10,13]],"date-time":"2025-10-13T09:33:49Z","timestamp":1760348029000},"page":"209-225","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Bioenergetic Profiling Applied to a Zebrafish Model of Neuronal Ceroid Lipofuscinosis, a Lysosomal Storage Disorder"],"prefix":"10.1007","author":[{"given":"Valentina","family":"Marchica","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily Malini","family":"Young","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nastassja","family":"Ramdeen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biggy","family":"Simbi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Russell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,10,14]]},"reference":[{"issue":"11","key":"15_CR1","doi-asserted-by":"publisher","first-page":"1882","DOI":"10.1016\/j.bbadis.2013.05.014","volume":"1832","author":"DN Palmer","year":"2013","unstructured":"Palmer DN, Barry LA, Tyynela J, Cooper JD (2013) NCL disease mechanisms. Biochim Biophys Acta 1832(11):1882\u20131893. https:\/\/doi.org\/10.1016\/j.bbadis.2013.05.014","journal-title":"Biochim Biophys Acta"},{"issue":"3","key":"15_CR2","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1038\/s41582-019-0138-8","volume":"15","author":"TB Johnson","year":"2019","unstructured":"Johnson TB, Cain JT, White KA et al (2019) Therapeutic landscape for Batten disease: current treatments and future prospects. Nat Rev Neurol 15(3):161\u2013178. https:\/\/doi.org\/10.1038\/s41582-019-0138-8","journal-title":"Nat Rev Neurol"},{"issue":"1","key":"15_CR3","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1002\/humu.21624","volume":"33","author":"M Kousi","year":"2012","unstructured":"Kousi M, Lehesjoki AE, Mole SE (2012) Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses. Hum Mutat 33(1):42\u201363. https:\/\/doi.org\/10.1002\/humu.21624","journal-title":"Hum Mutat"},{"issue":"8","key":"15_CR4","doi-asserted-by":"publisher","first-page":"582","DOI":"10.1016\/S2352-4642(18)30179-2","volume":"2","author":"M Nickel","year":"2018","unstructured":"Nickel M, Simonati A, Jacoby D et al (2018) Disease characteristics and progression in patients with late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease: an observational cohort study. Lancet Child Adolesc Health 2(8):582\u2013590. https:\/\/doi.org\/10.1016\/S2352-4642(18)30179-2","journal-title":"Lancet Child Adolesc Health"},{"issue":"6","key":"15_CR5","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1016\/s0197-0186(01)00128-0","volume":"40","author":"RD Jolly","year":"2002","unstructured":"Jolly RD, Brown S, Das AM, Walkley SU (2002) Mitochondrial dysfunction in the neuronal ceroid-lipofuscinoses (Batten disease). Neurochem Int 40(6):565\u2013571. https:\/\/doi.org\/10.1016\/s0197-0186(01)00128-0","journal-title":"Neurochem Int"},{"issue":"Suppl 2","key":"15_CR6","doi-asserted-by":"publisher","first-page":"81","DOI":"10.4137\/JEN.S25475","volume":"9","author":"RU Onyenwoke","year":"2015","unstructured":"Onyenwoke RU, Brenman JE (2015) Lysosomal storage diseases-regulating neurodegeneration. J Exp Neurosci 9(Suppl 2):81\u201391. https:\/\/doi.org\/10.4137\/JEN.S25475","journal-title":"J Exp Neurosci"},{"issue":"1","key":"15_CR7","doi-asserted-by":"publisher","DOI":"10.1016\/j.xpro.2020.100245","volume":"2","author":"X Gu","year":"2021","unstructured":"Gu X, Ma Y, Liu Y, Wan Q (2021) Measurement of mitochondrial respiration in adherent cells by Seahorse XF96 Cell Mito Stress Test. STAR Protoc 2(1):100245. https:\/\/doi.org\/10.1016\/j.xpro.2020.100245","journal-title":"STAR Protoc"},{"issue":"5","key":"15_CR8","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1007\/s10545-017-0048-0","volume":"40","author":"A Saffari","year":"2017","unstructured":"Saffari A, Kolker S, Hoffmann GF, Ebrahimi-Fakhari D (2017) Linking mitochondrial dysfunction to neurodegeneration in lysosomal storage diseases. J Inherit Metab Dis 40(5):631\u2013640. https:\/\/doi.org\/10.1007\/s10545-017-0048-0","journal-title":"J Inherit Metab Dis"},{"key":"15_CR9","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1016\/j.jprot.2015.03.038","volume":"123","author":"E Scifo","year":"2015","unstructured":"Scifo E, Szwajda A, Soliymani R et al (2015) Proteomic analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells. J Proteome 123:42\u201353. https:\/\/doi.org\/10.1016\/j.jprot.2015.03.038","journal-title":"J Proteome"},{"issue":"1","key":"15_CR10","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1007\/s12017-015-8382-6","volume":"18","author":"S Tikka","year":"2016","unstructured":"Tikka S, Monogioudi E, Gotsopoulos A et al (2016) Proteomic profiling in the brain of CLN1 disease model reveals affected functional modules. NeuroMolecular Med 18(1):109\u2013133. https:\/\/doi.org\/10.1007\/s12017-015-8382-6","journal-title":"NeuroMolecular Med"},{"key":"15_CR11","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1038\/s41420-020-0250-y","volume":"6","author":"S Doccini","year":"2020","unstructured":"Doccini S, Morani F, Nesti C et al (2020) Proteomic and functional analyses in disease models reveal CLN5 protein involvement in mitochondrial dysfunction. Cell Death Discov 6:18. https:\/\/doi.org\/10.1038\/s41420-020-0250-y","journal-title":"Cell Death Discov"},{"issue":"1","key":"15_CR12","doi-asserted-by":"publisher","first-page":"536","DOI":"10.1038\/s41467-022-28191-1","volume":"13","author":"I Lopez-Fabuel","year":"2022","unstructured":"Lopez-Fabuel I, Garcia-Macia M, Buondelmonte C et al (2022) Aberrant upregulation of the glycolytic enzyme PFKFB3 in CLN7 neuronal ceroid lipofuscinosis. Nat Commun 13(1):536. https:\/\/doi.org\/10.1038\/s41467-022-28191-1","journal-title":"Nat Commun"},{"key":"15_CR13","doi-asserted-by":"publisher","first-page":"266","DOI":"10.3389\/fnmol.2017.00266","volume":"10","author":"F Pezzini","year":"2017","unstructured":"Pezzini F, Bianchi M, Benfatto S et al (2017) The networks of genes encoding palmitoylated proteins in axonal and synaptic compartments are affected in PPT1 overexpressing neuronal-like cells. Front Mol Neurosci 10:266. https:\/\/doi.org\/10.3389\/fnmol.2017.00266","journal-title":"Front Mol Neurosci"},{"issue":"1\u20132","key":"15_CR14","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1016\/j.bbrc.2011.11.016","volume":"416","author":"F Pezzini","year":"2011","unstructured":"Pezzini F, Gismondi F, Tessa A et al (2011) Involvement of the mitochondrial compartment in human NCL fibroblasts. Biochem Biophys Res Commun 416(1\u20132):159\u2013164. https:\/\/doi.org\/10.1016\/j.bbrc.2011.11.016","journal-title":"Biochem Biophys Res Commun"},{"issue":"5","key":"15_CR15","doi-asserted-by":"publisher","first-page":"612","DOI":"10.1111\/jnc.14545","volume":"148","author":"ME Bosch","year":"2019","unstructured":"Bosch ME, Kielian T (2019) Astrocytes in juvenile neuronal ceroid lipofuscinosis (CLN3) display metabolic and calcium signaling abnormalities. J Neurochem 148(5):612\u2013624. https:\/\/doi.org\/10.1111\/jnc.14545","journal-title":"J Neurochem"},{"issue":"10","key":"15_CR16","doi-asserted-by":"publisher","DOI":"10.15252\/embr.202256380","volume":"24","author":"BR Desousa","year":"2023","unstructured":"Desousa BR, Kim KK, Jones AE et al (2023) Calculation of ATP production rates using the Seahorse XF Analyzer. EMBO Rep 24(10):e56380. https:\/\/doi.org\/10.15252\/embr.202256380","journal-title":"EMBO Rep"},{"issue":"Pt 5","key":"15_CR17","doi-asserted-by":"publisher","first-page":"1488","DOI":"10.1093\/brain\/awt043","volume":"136","author":"F Mahmood","year":"2013","unstructured":"Mahmood F, Fu S, Cooke J et al (2013) A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation. Brain 136(Pt 5):1488\u20131507. https:\/\/doi.org\/10.1093\/brain\/awt043","journal-title":"Brain"},{"issue":"6","key":"15_CR18","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0157365","volume":"11","author":"K Wager","year":"2016","unstructured":"Wager K, Zdebik AA, Fu S et al (2016) Neurodegeneration and epilepsy in a zebrafish model of CLN3 disease (Batten disease). PLoS One 11(6):e0157365. https:\/\/doi.org\/10.1371\/journal.pone.0157365","journal-title":"PLoS One"},{"key":"15_CR19","doi-asserted-by":"publisher","unstructured":"Crouzier L, Denus M, Richard EM et al (2021) Sigma-1 receptor is critical for mitochondrial activity and unfolded protein response in larval zebrafish. Int J Mol Sci 22(20). https:\/\/doi.org\/10.3390\/ijms222011049","DOI":"10.3390\/ijms222011049"},{"issue":"3","key":"15_CR20","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1080\/10495398.2016.1147455","volume":"27","author":"A Kumar","year":"2016","unstructured":"Kumar A, Singh N, Goswami M et al (2016) Establishment and characterization of a new muscle cell line of zebrafish (Danio rerio) as an in vitro model for gene expression studies. Anim Biotechnol 27(3):166\u2013173. https:\/\/doi.org\/10.1080\/10495398.2016.1147455","journal-title":"Anim Biotechnol"},{"key":"15_CR21","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2021.746367","volume":"12","author":"E Rollwitz","year":"2021","unstructured":"Rollwitz E, Jastroch M (2021) Plate-based respirometry to assess thermal sensitivity of zebrafish embryo bioenergetics in situ. Front Physiol 12:746367. https:\/\/doi.org\/10.3389\/fphys.2021.746367","journal-title":"Front Physiol"},{"issue":"4","key":"15_CR22","doi-asserted-by":"publisher","first-page":"393","DOI":"10.30802\/AALAS-JAALAS-19-000067","volume":"59","author":"EM Katz","year":"2020","unstructured":"Katz EM, Chu DK, Casey KM et al (2020) The stability and efficacy of tricaine methanesulfonate (MS222) solution after long-term storage. J Am Assoc Lab Anim Sci 59(4):393\u2013400. https:\/\/doi.org\/10.30802\/AALAS-JAALAS-19-000067","journal-title":"J Am Assoc Lab Anim Sci"}],"container-title":["Methods in Molecular Biology","Lysosomes"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/978-1-0716-4844-5_15","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T18:48:25Z","timestamp":1779302905000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/978-1-0716-4844-5_15"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,14]]},"ISBN":["9781071648438","9781071648445"],"references-count":22,"URL":"https:\/\/doi.org\/10.1007\/978-1-0716-4844-5_15","relation":{},"ISSN":["1064-3745","1940-6029"],"issn-type":[{"value":"1064-3745","type":"print"},{"value":"1940-6029","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,14]]},"assertion":[{"value":"14 October 2025","order":1,"name":"first_online","label":"First Online","group":{"name":"ChapterHistory","label":"Chapter History"}}]}}