Abstract:
:Regulatory factor X (Rfx) homologs regulate the transcription of genes necessary for ciliogenesis in invertebrates and vertebrates. Primary cilia are necessary for Hedgehog signaling and regulation of the activity of the transcriptional regulators known as Gli proteins, which are targets of Hedgehog signaling. Here, we describe an Rfx4(L298P) mouse mutant with distinct dorsoventral patterning defects in the ventral spinal cord and telencephalon due to aberrant Sonic hedgehog (Shh) signaling and Gli3 activity. We find that Ift172, which encodes an intraflagellar transport protein necessary for ciliogenesis, is a direct transcriptional target of Rfx4, and the decrease in its expression in the developing telencephalon and spinal cord of Rfx4(L298P) mutants correlates with defects in patterning and cilia formation. Our data indicate that Rfx4 is a regionally specific transcriptional regulator of ciliogenesis and thus is also a regionally specific modulator of Shh signaling during development of the central nervous system.
journal_name
Sci Signaljournal_title
Science signalingauthors
Ashique AM,Choe Y,Karlen M,May SR,Phamluong K,Solloway MJ,Ericson J,Peterson ASdoi
10.1126/scisignal.2000602subject
Has Abstractpub_date
2009-11-03 00:00:00pages
ra70issue
95eissn
1945-0877issn
1937-9145pii
2/95/ra70journal_volume
2pub_type
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