{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T22:03:42Z","timestamp":1790978622064,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2005,11,8]],"date-time":"2005-11-08T00:00:00Z","timestamp":1131408000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2005,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We recently demonstrated that calcium homeostasis is altered in mouse models of two sphingolipid storage diseases, Gaucher and Sandhoff diseases, owing to modulation of the activities of a calcium\u2010release channel (the ryanodine receptor) and of the sarco\/endoplasmic reticulum Ca<jats:sup>2+<\/jats:sup>\u2010ATPase (SERCA) respectively, by the accumulating sphingolipids. We now demonstrate that calcium homeostasis is also altered in a mouse model of Niemann\u2013Pick A disease, the acid sphingomyelinase (A\u2010SMase)\u2010deficient mouse (ASM\u2013\/\u2013), with reduced rates of calcium uptake via SERCA in the cerebellum of 6\u20137\u2010month\u2010old mice. However, the mechanism responsible for defective calcium homeostasis is completely different from that observed in the other two disease models. Thus, levels of SERCA expression are significantly reduced in the ASM\u2013\/\u2013 cerebellum by 6\u20137\u2003months of age, immediately before death of the mice, as are levels of the inositol 1,4,5\u2010triphosphate receptor (IP<jats:sub>3<\/jats:sub>R), the major calcium\u2010release channel in the cerebellum. Systematic analyses of the time course of loss of SERCA and IP<jats:sub>3<\/jats:sub>R expression revealed that loss of the IP<jats:sub>3<\/jats:sub>R preceeded that of SERCA, with essentially no IP<jats:sub>3<\/jats:sub>R remaining by 4\u2003months of age, whereas SERCA was still present even after 6\u2003months. Expression of zebrin II (aldolase C), a protein found in about half of the Purkinje cells in the adult mouse cerebellum, was essentially unchanged during development. We discuss possible pathological mechanisms related to calcium dysfunction that may cause Purkinje cell degeneration, and as a result, the onset of neuropathology in Niemann\u2013Pick A disease.<\/jats:p>","DOI":"10.1111\/j.1471-4159.2005.03534.x","type":"journal-article","created":{"date-parts":[[2005,11,8]],"date-time":"2005-11-08T10:51:33Z","timestamp":1131447093000},"page":"1619-1628","source":"Crossref","is-referenced-by-count":54,"title":["Defective calcium homeostasis in the cerebellum in a mouse model of Niemann\u2013Pick A disease"],"prefix":"10.1111","volume":"95","author":[{"given":"Luba","family":"Ginzburg","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony H.","family":"Futerman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,11,8]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.35.20459"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrm1155"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1139\/y59-099"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(73)90363-X"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2004.02457.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000042958.22066.6c"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0306-4522(84)90193-3"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrm1423"},{"key":"e_1_2_7_10_1","doi-asserted-by":"crossref","first-page":"11\u2003075","DOI":"10.1016\/S0021-9258(18)37923-7","article-title":"Competitive, reversible, and potent antagonism of inositol 1,4,5\u2010trisphosphate\u2010activated calcium release by heparin","volume":"263","author":"Ghosh T. K.","year":"1988","journal-title":"J. Biol. Chem."},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcdb.2004.03.003"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02736761"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj20030110"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0795-288"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.31.21673"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/72101"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M300212200"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20030613"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1203-1041"},{"key":"e_1_2_7_20_1","first-page":"144","article-title":"Rapidly exchanging Ca2+ stores: ubiquitous partners of surface channels in neurons","volume":"17","author":"Meldolesi J.","year":"2002","journal-title":"News Physiol. Sci."},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-1981(02)00154-3"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0143416002001884"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M302964200"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2004.09.004"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:GLYC.0000046272.38480.ef"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1022501702403"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0301-0082(03)00114-X"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.0953-816x.2001.01564.x"},{"key":"e_1_2_7_29_1","first-page":"3589","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Schuchman E. H.","year":"2001"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh268"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2004.08.029"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-6147(98)01184-5"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.50.1.106"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.113.4.779"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.9.5020"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.7189903"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1995.269.3.C775"}],"container-title":["Journal of Neurochemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1471-4159.2005.03534.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1471-4159.2005.03534.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T19:07:21Z","timestamp":1695841641000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1471-4159.2005.03534.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,11,8]]},"references-count":36,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2005,12]]}},"alternative-id":["10.1111\/j.1471-4159.2005.03534.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1471-4159.2005.03534.x","archive":["Portico"],"relation":{},"ISSN":["0022-3042","1471-4159"],"issn-type":[{"value":"0022-3042","type":"print"},{"value":"1471-4159","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,11,8]]}}}