Disruption of planar cell polarity activity leads to developmental biliary defects

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Disruption of planar cell polarity activity leads to developmental biliary defects.

Planar cell polarity (PCP) establishes polarity within an epithelial sheet. Defects in PCP are associated with developmental defects involving directional cell growth, including defects in kidney tubule elongation that lead to formation of kidney cysts. Given the strong association between kidney cyst formation and developmental biliary defects in patients and in animal models, we investigated ...

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The Drosophila scribble gene regulates apical-basal polarity and is implicated in control of cellular architecture and cell growth control. Mutations in mammalian Scrib (circletail; Crc mutant) also result in abnormalities suggestive of roles in planar cell polarity regulation. We show that Crc mutants develop heart malformations and cardiomyopathy attributable to abnormalities in cardiomyocyte...

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Planar cell polarity, ciliogenesis and neural tube defects.

Cilia are microtubule-based protrusions that are found on the surface of most vertebrate cells. Long studied by cell biologists, these organelles have recently caught the attention of developmental biologists and human geneticists. In this review, I will discuss recent findings suggesting a link between cilia and the planar cell polarity signaling cascade. In particular, I will focus on how thi...

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ژورنال

عنوان ژورنال: Developmental Biology

سال: 2011

ISSN: 0012-1606

DOI: 10.1016/j.ydbio.2010.12.041