{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T10:31:45Z","timestamp":1722508305853},"reference-count":48,"publisher":"Walter de Gruyter GmbH","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,7,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n<jats:sec id=\"j_jpem-2018-0540_s_999\">\n<jats:title id=\"d3356547e36694\">Background<\/jats:title>\n<jats:p>Neonatal adrenoleukodystrophy (n-ALD) and X-linked ALD (X-ALD) patients present with demyelination, poor growth and progressive mental retardation. Growth factors are known to play a vital role in the development of children.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2018-0540_s_998\">\n<jats:title id=\"d3356547e36702\">Objective<\/jats:title>\n<jats:p>To examine the mitogenic activity of various growth factors in skin fibroblasts from n-ALD and X-ALD patients.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2018-0540_s_997\">\n<jats:title id=\"d3356547e36710\">Methods<\/jats:title>\n<jats:p>Skin fibroblast cultures from n-ALD and X-ALD patients, and controls were treated with 50 ng\/mL of platelet-derived growth factor (PDGF), basic fibroblast growth factor (bFGF) or insulin-like growth factor-1 (IGF-1) to examine DNA synthesis by 5-bromo-2\u2032-deoxyuridine (BrdU) incorporation. Expression of receptors for PDGF, bFGF and IGF-1 was measured by western blotting. Serum levels of IGF-1 were assayed by enzyme-linked immunosorbent assay (ELISA).<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2018-0540_s_996\">\n<jats:title id=\"d3356547e36718\">Results<\/jats:title>\n<jats:p>Fibroblasts from n-ALD and X-ALD patients had significantly (p\u2009&lt;\u20090.01) less BrdU incorporation in response to fetal bovine serum (FBS). The mitogenic effect of PDGF, bFGF and IGF-1 was significantly lower in n-ALD as compared to control and X-ALD cells. X-ALD cells showed significant impairment in IGF-1-induced DNA synthesis. Expression of the FGF receptor (FGF-R) was significantly reduced in n-ALD cells. PDGF receptor remained unaffected, and IGF-1 receptor (IGF-1R) expression and serum IGF-1 levels were significantly (p\u2009&lt;\u20090.01) reduced in n-ALD and X-ALD patients as compared to controls.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2018-0540_s_995\">\n<jats:title id=\"d3356547e36727\">Conclusions<\/jats:title>\n<jats:p>Growth factor activity differs in n-ALD and X-ALD patients, with marked impairment of IGF-1 function through receptor down-regulation.<\/jats:p><\/jats:sec>","DOI":"10.1515\/jpem-2018-0540","type":"journal-article","created":{"date-parts":[[2019,6,13]],"date-time":"2019-06-13T09:09:32Z","timestamp":1560416972000},"page":"733-738","source":"Crossref","is-referenced-by-count":3,"title":["Selective receptor-mediated impairment of growth factor activity in neonatal- and X-linked adrenoleukodystrophy patients"],"prefix":"10.1515","volume":"32","author":[{"given":"Mazen","family":"Al-Essa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gursev S.","family":"Dhaunsi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref271","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1056\/NEJM199702273360907","article-title":"Seminars in medicine of the Beth Israel Deaconess Medical Center. 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