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NTDs' pathogenesis has been linked to genes involved in folate metabolism, consistent with an epidemiologic evidence that 70% of NTDs can be prevented by maternal periconceptional supplementation. However, polymorphisms in such genes are not linked in all populations, suggesting that other genetic factors and environmental factors could be involved. Animal models have provided crucial mechanistic information and possible candidate genes to explain susceptibility to NTDs. A crucial role has been assigned to the planar cell polarity (PCP) pathway, a highly conserved, non\u2010canonical Wnt\u2010frizzled\u2010dishevelled signaling cascade that plays a key role in establishing and maintaining polarity in the plane of the epithelium and in the process of convergent extension during gastrulation and neurulation in vertebrates. The Loop\u2010tail (Lp) mouse that develops craniorachischisis carry missense mutations in the PCP core gene Vangl2, that is the mammalian homolog of the Drosophila Strabismus\/Van gogh (Stbm\/Vang). The presence of mutations in human<jats:italic>VANGL1<\/jats:italic>and<jats:italic>VANGL2<\/jats:italic>genes encourages us to extend the investigation to other PCP genes that, with VANGL, play an essential role in neurulation during development.<\/jats:p>","DOI":"10.1002\/biof.170","type":"journal-article","created":{"date-parts":[[2011,6,15]],"date-time":"2011-06-15T09:59:55Z","timestamp":1308131995000},"page":"261-268","source":"Crossref","is-referenced-by-count":43,"title":["Human neural tube defects: Genetic causes and prevention"],"prefix":"10.1002","volume":"37","author":[{"given":"Patrizia","family":"De Marco","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Merello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armando","family":"Cama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoha","family":"Kibar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Capra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,6,14]]},"reference":[{"key":"e_1_2_4_2_2","unstructured":"EUROCAT Working Group. 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