{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T20:01:52Z","timestamp":1760644912566},"reference-count":55,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[1993,5,8]],"date-time":"1993-05-08T00:00:00Z","timestamp":736819200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1994,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>A system based on the ability of cells to oxidize very long\u2010chain fatty acids (VLCFA) was developed to select<jats:italic>in vitro<\/jats:italic> normal human fibroblasts from fibroblasts of patients suffering from peroxisomal disorders with multienzymatic deficiencies: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease (IRD). Cells treated with various concentrations of erucic acid (C<jats:sub>22:1 n\u20109<\/jats:sub>) revealed an enhanced toxicity of this fatty acid for the fibroblasts of patients compared with normal cells. This differential toxicity is correlated with variable accumulations of C<jats:sub>22:1 n\u20109<\/jats:sub> and the absence of \u03b2\u2010oxidation products in the mutants.<\/jats:p><jats:p>Revertants from clonal IRD cell lines were isolated in the selective medium at frequencies ranging from 3\u00d710<jats:sup>\u22127<\/jats:sup> to 4\u00d710<jats:sup>\u22126<\/jats:sup> depending on the line. After six weeks of growth in the absence of selective pressure, the variants exhibited a resistance level to C<jats:sub>22:1 n\u20109<\/jats:sub> identical to that of normal cells. Furthermore, \u03b2\u2010oxidation of VLCFA is re\u2010established in these selected cells as well as dihydroxyacetone phosphate acyltransferase activity. Immunoblot experiments also demonstrated a restored pattern of acyl\u2010CoA oxidase molecular forms. Last, immunofluorescence studies revealed the presence of cytoplasmic structures that were absent in the original IRD cells. Thus, both the deficiencies in metabolic pathways and paucity of the organelle are at least partially corrected in the selected clones.<\/jats:p>","DOI":"10.1007\/bf00735394","type":"journal-article","created":{"date-parts":[[2004,12,3]],"date-time":"2004-12-03T13:40:11Z","timestamp":1102081211000},"page":"41-59","source":"Crossref","is-referenced-by-count":11,"title":["Resistance to erucic acid as a selectable marker for peroxisomal activity: Isolation of revertants of an infantile Refsum disease cell line"],"prefix":"10.1002","volume":"17","author":[{"given":"Bachir","family":"El Bioukar","sequence":"first","affiliation":[{"name":"INSERM U 347, Bic\u00eatre Hospital  Kremlin\u2010Bic\u00eatre 94 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Straehli","sequence":"additional","affiliation":[{"name":"INSERM U 347, Bic\u00eatre Hospital  Kremlin\u2010Bic\u00eatre 94 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K. Heng","family":"Ng","sequence":"additional","affiliation":[{"name":"INSERM U 347, Bic\u00eatre Hospital  Kremlin\u2010Bic\u00eatre 94 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. \u2010O.","family":"Rolland","sequence":"additional","affiliation":[{"name":"Service de Biochimie Debrousse Hospital  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Hashimoto","sequence":"additional","affiliation":[{"name":"Department of Biochemistry Shinohu University School of Medicine  Matsumoto, Nagano 390 Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. \u2010P.","family":"Carreau","sequence":"additional","affiliation":[{"name":"INSERM U 347, Bic\u00eatre Hospital  Kremlin\u2010Bic\u00eatre 94 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Deschatrette","sequence":"additional","affiliation":[{"name":"INSERM U 347, Bic\u00eatre Hospital  Kremlin\u2010Bic\u00eatre 94 France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1993,5,8]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.22.10084"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199003000-00023"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/290304a0"},{"key":"e_1_2_1_5_1","first-page":"402","article-title":"A familial syndrome of multiple congenital defects","volume":"114","author":"Bowen P","year":"1964","journal-title":"Bull. Johns Hopkins Hosp"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113510"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9673(00)88356-9"},{"key":"e_1_2_1_8_1","first-page":"1973","article-title":"M\u00e9tabolisme de l'acide \u00e9rucique. I \u2014 Conversion en acide ol\u00e9ique","volume":"50","author":"Carreau JP","year":"1968","journal-title":"Bull Soc Chim Biol"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02534012"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(89)90067-2"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.62.3.852"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-3002(60)91338-X"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0300-9084(75)80286-0"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199102000-00007"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0492-16"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1973.tb02881.x"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(89)91587-8"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)86607-3"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115509"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(89)90415-3"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)48390-1"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)37854-8"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)63419-2"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/227680a0"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(91)90144-7"},{"key":"e_1_2_1_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)34897-4"},{"key":"e_1_2_1_27_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.01.110185.002421"},{"key":"e_1_2_1_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0300-9084(74)80078-7"},{"key":"e_1_2_1_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_1_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(05)80218-9"},{"key":"e_1_2_1_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-9120(91)80009-R"},{"key":"e_1_2_1_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)34409-5"},{"key":"e_1_2_1_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-4827(92)90278-G"},{"key":"e_1_2_1_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02533382"},{"key":"e_1_2_1_35_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jbchem.a132918"},{"key":"e_1_2_1_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441753"},{"key":"e_1_2_1_37_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799455"},{"key":"e_1_2_1_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441598"},{"key":"e_1_2_1_39_1","first-page":"537","volume-title":"Fatty Acid Oxidation: Clinical, Biochemical and Molecular Aspects","author":"Poll\u2010Th\u00e9 BT","year":"1990"},{"key":"e_1_2_1_40_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.12.1606"},{"key":"e_1_2_1_41_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320250210"},{"key":"e_1_2_1_42_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198907000-00019"},{"key":"e_1_2_1_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)81544-2"},{"key":"e_1_2_1_44_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.83.16.6156"},{"key":"e_1_2_1_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799998"},{"key":"e_1_2_1_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(84)91430-X"},{"key":"e_1_2_1_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(87)90133-0"},{"key":"e_1_2_1_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(75)80083-0"},{"key":"e_1_2_1_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)45395-1"},{"key":"e_1_2_1_50_1","doi-asserted-by":"publisher","DOI":"10.1038\/350077a0"},{"key":"e_1_2_1_51_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00685001"},{"key":"e_1_2_1_52_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800588"},{"key":"e_1_2_1_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(88)90203-1"},{"key":"e_1_2_1_54_1","first-page":"407","article-title":"Presence of peroxisomal membrane proteins in liver and fibroblasts from patients with Zellweger syndrome and related disorders: evidence for the existence of peroxisomal ghosts","volume":"50","author":"Wiemer EAC","year":"1989","journal-title":"Eur J Cell Biol"},{"key":"e_1_2_1_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(91)90041-7"},{"key":"e_1_2_1_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)38000-1"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF00735394","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T04:40:48Z","timestamp":1694925648000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF00735394"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1993,5,8]]},"references-count":55,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1994,1]]}},"alternative-id":["10.1007\/BF00735394"],"URL":"https:\/\/doi.org\/10.1007\/bf00735394","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1993,5,8]]}}}