{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:25:24Z","timestamp":1786976724771,"version":"build-2736575974"},"reference-count":14,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2008,5,1]],"date-time":"2008-05-01T00:00:00Z","timestamp":1209600000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt B"],"published-print":{"date-parts":[[2008,10,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Lack of production of the Fragile X Mental Retardation Protein (FMRP) leads to changes in dendritic morphology and resultant cognitive and behavioral manifestations characteristic of individuals with Fragile X syndrome (FXS). FMRP is an RNA\u2010binding protein that is believed to regulate the translation of a large number (probably over 100) of other proteins, leading to a complex and variable set of symptoms in FXS. In a mouse model of FXS, we previously observed delayed initiation of synaptically localized protein synthesis in response to neurotransmitter stimulation, as compared to wild\u2010type mice. We now likewise have observed delayed early\u2010phase phosphorylation of extracellular\u2010signal regulated kinase (ERK), a nodal point for cell signaling cascades, in both neurons and thymocytes of fmr\u20101 KO mice. We further report that early\u2010phase kinetics of ERK activation in lymphocytes from human peripheral blood is delayed in a cohort of individuals with FXS, relative to normlal controls, suggesting a potential biomarker to measure metabolic status of disease for individuals with FXS. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.b.30765","type":"journal-article","created":{"date-parts":[[2008,5,1]],"date-time":"2008-05-01T14:23:36Z","timestamp":1209651816000},"page":"1253-1257","source":"Crossref","is-referenced-by-count":60,"title":["Early\u2010phase ERK activation as a biomarker for metabolic status in fragile X syndrome"],"prefix":"10.1002","volume":"147B","author":[{"given":"Ning","family":"Weng","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan Jeanne","family":"Weiler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison","family":"Sumis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Berry\u2010Kravis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William T.","family":"Greenough","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrdd.20007"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1054\/cupe.2002.0305"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1089\/cap.2006.16.525"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2005.03456.x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.a.20167"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pnpbp.2004.01.009"},{"key":"e_1_2_6_8_1","doi-asserted-by":"crossref","first-page":"287","DOI":"10.56021\/9780801868436","volume-title":"Fragile X Syndrome: Diagnosis, Treatment, and Research","author":"Hagerman RJ","year":"2002"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.10216"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsych.2006.02.027"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(03)00034-5"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M401761200"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/cyto.b.20092"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ridd.2006.09.001"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0407533101"}],"container-title":["American Journal of Medical Genetics Part B: Neuropsychiatric Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.b.30765","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.b.30765","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,11]],"date-time":"2023-10-11T01:21:42Z","timestamp":1696987302000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.b.30765"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,5]]},"references-count":14,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2008,10,5]]}},"alternative-id":["10.1002\/ajmg.b.30765"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.b.30765","archive":["Portico"],"relation":{},"ISSN":["1552-4841","1552-485X"],"issn-type":[{"value":"1552-4841","type":"print"},{"value":"1552-485X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,5]]}}}