Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closure

التفاصيل البيبلوغرافية
العنوان: Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closure
المؤلفون: Sarah Sarsfield, Yanshu Wang, Randy Schekman, Janna Merte, Devon Jensen, David D. Ginty, Kevin M. Wright
المصدر: Nature cell biology
بيانات النشر: Springer Science and Business Media LLC, 2009.
سنة النشر: 2009
مصطلحات موضوعية: Male, Neural Tube, Vesicular Transport Proteins, Fluorescent Antibody Technique, Nerve Tissue Proteins, SEC24B, Biology, Endoplasmic Reticulum, Article, Immunoenzyme Techniques, Mice, Mice, Neurologic Mutants, 03 medical and health sciences, 0302 clinical medicine, Cell polarity, Craniorachischisis, medicine, Animals, Immunoprecipitation, Neural Tube Defects, COPII, 030304 developmental biology, Mice, Inbred C3H, 0303 health sciences, Convergent extension, Endoplasmic reticulum, Neural tube, Cell Polarity, Cell Biology, COP-Coated Vesicles, Embryo, Mammalian, Cell biology, Mice, Inbred C57BL, medicine.anatomical_structure, Spinal Cord, 030217 neurology & neurosurgery, Hair, Signal Transduction
الوصف: Craniorachischisis is a rare but severe birth defect that results in a completely open neural tube. Mouse mutants in planar cell polarity (PCP) signalling components have deficits in the morphological movements of convergent extension that result in craniorachischisis. Using a forward genetic screen in mice, we identified Sec24b, a cargo-sorting member of the core complex of the endoplasmic reticulum (ER)-to-Golgi transport vesicle COPII, as critical for neural tube closure. Sec24bY613 mutant mice exhibit craniorachischisis, deficiencies in convergent extension and other PCP-related phenotypes. Vangl2, a key component of the PCP-signalling pathway critical for convergent extension, is selectively sorted into COPII vesicles by Sec24b. Moreover, Sec24bY613 genetically interacts with a loss-of-function Vangl2 allele (Vangl2LP), causing a marked increase in the prevalence of spina bifida. Interestingly, the Vangl2 looptail point mutants Vangl2D255E and Vangl2S464N, known to cause defects in convergent extension, fail to sort into COPII vesicles and are trapped in the ER. Thus, during COPII vesicle formation, Sec24b shows cargo specificity for a core PCP component, Vangl2, of which proper ER-to-Golgi transport is essential for the establishment of PCP, convergent extension and closure of the neural tube.
تدمد: 1476-4679
1465-7392
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5b82962c125e2a46e46bb887427f32a1Test
https://doi.org/10.1038/ncb2002Test
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....5b82962c125e2a46e46bb887427f32a1
قاعدة البيانات: OpenAIRE
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