Planar cell polarity defects and defective Vangl2 trafficking in mutants for the COPII gene Sec24b.

C. Wansleeben, H. Feitsma, M. Montcouquiol, C. Kroon, E. Cuppen, F. Meijlink
Development. 2010-03-09; 137(7): 1067-1073
DOI: 10.1242/dev.041434

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1. Development. 2010 Apr;137(7):1067-73. doi: 10.1242/dev.041434.

Planar cell polarity defects and defective Vangl2 trafficking in mutants for the
COPII gene Sec24b.

Wansleeben C(1), Feitsma H, Montcouquiol M, Kroon C, Cuppen E, Meijlink F.

Author information:
(1)Hubrecht Institute, KNAW & University Medical Center Utrecht, Uppsalalaan 8,
3584 CT Utrecht, The Netherlands.

Among the cellular properties that are essential for the organization of tissues
during animal development, the importance of cell polarity in the plane of
epithelial sheets has become increasingly clear in the past decades. Planar cell
polarity (PCP) signaling in vertebrates has indispensable roles in many aspects
of their development, in particular, controlling alignment of various types of
epithelial cells. Disrupted PCP has been linked to developmental defects in
animals and to human pathology. Neural tube closure defects (NTD) and
disorganization of the mechanosensory cells of the organ of Corti are commonly
known consequences of disturbed PCP signaling in mammals. We report here a
typical PCP phenotype in a mouse mutant for the Sec24b gene, including the severe
NTD craniorachischisis, abnormal arrangement of outflow tract vessels and
disturbed development of the cochlea. In addition, we observed genetic
interaction between Sec24b and the known PCP gene, scribble. Sec24b is a
component of the COPII coat protein complex that is part of the endoplasmic
reticulum (ER)-derived transport vesicles. Sec24 isoforms are thought to be
directly involved in cargo selection, and we present evidence that Sec24b
deficiency specifically affects transport of the PCP core protein Vangl2, based
on experiments in embryos and in cultured primary cells.

DOI: 10.1242/dev.041434
PMID: 20215345 [Indexed for MEDLINE]


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