TY - JOUR
T1 - Planar cell polarity effector gene Fuzzy regulates cilia formation and hedgehog signal transduction in mouse
AU - Heydeck, Westley
AU - Zeng, Huiqing
AU - Liu, Aimin
PY - 2009/12
Y1 - 2009/12
N2 - Precise planar cell polarity (PCP) is critical for the development of multiple organ systems in animals. A group of core-PCP proteins are recognized to play crucial roles in convergent extension and other PCP-related processes in mammals. However, the functions of another group of PCP-regulating proteins, the PCP-effector proteins, are yet to be fully studied. In this study, the generation and characterization of a mouse mutant for the PCP effector gene Fuzzy (Fuz) is reported. Fuz homozygous mutants are embryonically lethal, with multiple defects including neural tube defects, abnormal dorsal/ventral patterning of the spinal cord, and defective anterior/posterior patterning of the limb buds. Fuz mutants also exhibit abnormal Hedgehog (Hh) signaling and inefficient proteolytic processing of Gli3. Finally, a significant decrease in cilia was found in Fuz homozygous mutants. In conclusion, Fuz plays an important role in cilia formation, Hh signal transduction, and embryonic development in mammals.
AB - Precise planar cell polarity (PCP) is critical for the development of multiple organ systems in animals. A group of core-PCP proteins are recognized to play crucial roles in convergent extension and other PCP-related processes in mammals. However, the functions of another group of PCP-regulating proteins, the PCP-effector proteins, are yet to be fully studied. In this study, the generation and characterization of a mouse mutant for the PCP effector gene Fuzzy (Fuz) is reported. Fuz homozygous mutants are embryonically lethal, with multiple defects including neural tube defects, abnormal dorsal/ventral patterning of the spinal cord, and defective anterior/posterior patterning of the limb buds. Fuz mutants also exhibit abnormal Hedgehog (Hh) signaling and inefficient proteolytic processing of Gli3. Finally, a significant decrease in cilia was found in Fuz homozygous mutants. In conclusion, Fuz plays an important role in cilia formation, Hh signal transduction, and embryonic development in mammals.
UR - http://www.scopus.com/inward/record.url?scp=70949085969&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=70949085969&partnerID=8YFLogxK
U2 - 10.1002/dvdy.22130
DO - 10.1002/dvdy.22130
M3 - Article
C2 - 19877275
AN - SCOPUS:70949085969
SN - 1058-8388
VL - 238
SP - 3035
EP - 3042
JO - Developmental Dynamics
JF - Developmental Dynamics
IS - 12
ER -