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Among a variety of embryological toxins that can induce HPE, inhibitors, and other pertubations of cholesterol biosynthesis have been shown to be important factors, most likely because cholesterol is required in the Sonic hedgehog signaling cascade. Decreased levels of maternal cholesterol during pregnancy increase the risk for preterm delivery, but they are not associated with congenital malformations. However, if the fetus is affected by an inborn error of endogenous cholesterol synthesis, a reduction of maternal cholesterol concentration and cholesterol transport over the placenta aggravates the phenotypic expression. Exposure to lipophilic statins in early pregnancy may be associated with a substantial risk for structural CNS defects. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.c.30243","type":"journal-article","created":{"date-parts":[[2010,1,26]],"date-time":"2010-01-26T22:18:52Z","timestamp":1264544332000},"page":"102-108","source":"Crossref","is-referenced-by-count":41,"title":["Abnormal sterol metabolism in holoprosencephaly"],"prefix":"10.1002","volume":"154C","author":[{"given":"Dorothea","family":"Haas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maximilian","family":"Muenke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,1,26]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"1164","article-title":"A congenital cyclopian\u2010type malformation in lambs induced by maternal ingestion of a range plant, veratum californicum","volume":"24","author":"Binns W","year":"1963","journal-title":"Am J Vet Res"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/2484"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1025302"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/383407a0"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1134"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0602852103"},{"key":"e_1_2_8_8_1","doi-asserted-by":"crossref","first-page":"8484","DOI":"10.1016\/S0021-9258(19)38914-8","article-title":"Zymosterol is located in the plasma membrane of cultured human fibroblasts","volume":"265","author":"Echevarria F","year":"1990","journal-title":"J Biol Chem"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200404083501524"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30386"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-1939"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.14.8181"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/76074"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.047258"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.125.18.3553"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/humupd\/6.3.268"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/tera.1420010103"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.37.5.321"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1053\/plac.1999.0412"},{"key":"e_1_2_8_20_1","doi-asserted-by":"crossref","first-page":"4433","DOI":"10.1016\/S0021-9258(18)61210-4","article-title":"Cholesterol is not synthesized in membranes bearing 3\u2010hydroxy\u20103\u2010methylglutaryl coenzyme A reductase","volume":"262","author":"Lange Y","year":"1987","journal-title":"J Biol Chem"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn278"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9378(66)90416-9"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-1981(00)00148-7"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1086\/344412"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-002-0695-5"},{"key":"e_1_2_8_26_1","first-page":"6203","volume-title":"The metabolic and molecular bases of inherited disease. 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