{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,20]],"date-time":"2026-04-20T22:25:04Z","timestamp":1776723904786,"version":"3.51.2"},"reference-count":19,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2001,1,1]],"date-time":"2001-01-01T00:00:00Z","timestamp":978307200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European Journal of Paediatric Neurology"],"published-print":{"date-parts":[[2001,1]]},"DOI":"10.1053\/ejpn.2000.0451","type":"journal-article","created":{"date-parts":[[2006,12,14]],"date-time":"2006-12-14T07:10:57Z","timestamp":1166080257000},"page":"143-146","source":"Crossref","is-referenced-by-count":14,"special_numbering":"S1","title":["Altered levels of high-energy phosphate compoundsin fibroblasts from different forms of neuronal ceroid lipofuscinoses: further evidence for mitochondria) involvement"],"prefix":"10.1053","volume":"5","author":[{"given":"Anibh M.","family":"Das","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Remko Von","family":"Harlem","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miriam","family":"Feist","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"L\u00fccke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alfried","family":"Kohlschotter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1053\/ejpn.2000.0451_bib1","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1002\/ajmg.1320570219","article-title":"Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease","volume":"57","author":"March","year":"1995","journal-title":"Am J Med Genet"},{"key":"10.1053\/ejpn.2000.0451_bib2","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1002\/ajmg.1320570218","article-title":"Pathogenesis of brain dysfunction in Batten disease","volume":"57","author":"Walkley","year":"1995","journal-title":"Am J Med Genet"},{"key":"10.1053\/ejpn.2000.0451_bib3","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1007\/BF01273267","article-title":"Loss of pigment-laden stellate cells: a severe alteration of the isocortex in juvenile neuronal ceroid-lipofuscinosis","volume":"42","author":"Braak","year":"1978","journal-title":"Acta Neuropathol Berl"},{"key":"10.1053\/ejpn.2000.0451_bib4","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/BF00684808","article-title":"Pigmentoarchitectonic pathology of the isocortex in juvenile ceroid-lipofuscinosis: axonal enlargements in layer IIIab and cell loss in layer V","volume":"46","author":"Braak","year":"1979","journal-title":"Acta Neuropathol Berl"},{"key":"10.1053\/ejpn.2000.0451_bib5","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1111\/j.1750-3639.1998.tb00144.x","article-title":"Cellular pathology of lysosomal storage disorders","volume":"8","author":"Walkley","year":"1998","journal-title":"Brain Pathol"},{"key":"10.1053\/ejpn.2000.0451_bib6","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1136\/jnnp.53.12.1063","article-title":"Neuronal ceroid-lipofuscinosis: preferential metabolic alterations in thalamus and posterior association cortex demonstrated by PET","volume":"53","author":"De Volder","year":"1990","journal-title":"J Neurosurg Psychiat"},{"key":"10.1053\/ejpn.2000.0451_bib7","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1002\/ajmg.1320420428","article-title":"Mitochondrial ATP synthase subunit c storage in the ceroidlipofuscinoses (Batten disease)","volume":"42","author":"Palmer","year":"1992","journal-title":"Am J Med Genet"},{"key":"10.1053\/ejpn.2000.0451_bib8","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1007\/BF01799411","article-title":"Decreased activity of the mitochondrial ATP-synthase in fibroblasts from children with late-infantile and juvenile neuronal ceroid lipofuscinosis","volume":"19","author":"Das","year":"1996","journal-title":"J Inher Metab Dis"},{"key":"10.1053\/ejpn.2000.0451_bib9","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1006\/mgme.1999.2811","article-title":"Anomalies of mitochondrial ATP synthase regulation in four different types of neuronal ceroid lipofuscinosis","volume":"66","author":"Das","year":"1999","journal-title":"Mol Gen Metab"},{"key":"10.1053\/ejpn.2000.0451_bib10","series-title":"The Neuronal Ceroid Lipofuscinoses (Batten Disease)","first-page":"142","article-title":"Morphologic diagnostic considerations","author":"Lake","year":"1999"},{"key":"10.1053\/ejpn.2000.0451_bib11","series-title":"What is Controlling Life?","first-page":"141","article-title":"ATPsynthesis in permeabilized cells: assessment of the ATP\/O ratios in situ","author":"Ouhabi","year":"1994"},{"key":"10.1053\/ejpn.2000.0451_bib12","series-title":"Spectrophotometry and Spectrofluorimetry","first-page":"74","article-title":"Spectrophotometric assays","author":"Harris","year":"1988"},{"key":"10.1053\/ejpn.2000.0451_bib13","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/S0003-2697(76)80064-4","article-title":"Assay of proteins in the presence of interfering material","volume":"70","author":"Bensadoun","year":"1978","journal-title":"Anal Biochim"},{"key":"10.1053\/ejpn.2000.0451_bib14","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/0009-8981(86)90246-9","article-title":"Estimation of NADH oxidation in human skeletal muscle mitochondria","volume":"155","author":"Fischer","year":"1986","journal-title":"Clin Chim Acta"},{"key":"10.1053\/ejpn.2000.0451_bib15","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1006\/mgme.1999.2822","article-title":"Ion pores made of mitochondrial ATPsynthase subunit c in the neuronal plasma membrane and Batten disease","volume":"66","author":"McGeoch","year":"1999","journal-title":"Mol Genet Metab"},{"key":"10.1053\/ejpn.2000.0451_bib16","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/S0005-2760(96)00153-1","article-title":"Abnormalities in mitochondria-associated membranes and phospholipid biosynthetic enzymes in the mnd\/mnd mouse model of neuronal ceroid lipofuscinosis","volume":"1344","author":"Vance","year":"1997","journal-title":"Biochim Biophys Acta"},{"key":"10.1053\/ejpn.2000.0451_bib17","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/0005-2728(88)90051-5","article-title":"The binding and release of the inhibitor protein are governed independently by ATP and membrane potential in ox heart submitochondrial particles","volume":"933","author":"Lippe","year":"1988","journal-title":"Biochim Biophys Acta"},{"key":"10.1053\/ejpn.2000.0451_bib18","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1023\/A:1021036506299","article-title":"Altered mitochondrial function in canine ceroid-lipofuscinosis","volume":"23","author":"Siakotos","year":"1998","journal-title":"Neurochem Res"},{"key":"10.1053\/ejpn.2000.0451_bib19","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1002\/ana.410310202","article-title":"Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative diseases?","volume":"31","author":"Beal","year":"1992","journal-title":"Ann Neurol"}],"container-title":["European Journal of Paediatric Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1090379805800273?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1090379805800273?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,1,10]],"date-time":"2019-01-10T16:56:54Z","timestamp":1547139414000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1090379805800273"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,1]]},"references-count":19,"alternative-id":["S1090379805800273"],"URL":"https:\/\/doi.org\/10.1053\/ejpn.2000.0451","relation":{},"ISSN":["1090-3798"],"issn-type":[{"value":"1090-3798","type":"print"}],"subject":[],"published":{"date-parts":[[2001,1]]}}}