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XB-ART-48815
Proc Natl Acad Sci U S A April 29, 2014; 111 (17): 6329-34.

Sp8 regulates inner ear development.

Chung HA , Medina-Ruiz S , Harland RM .


Abstract
A forward genetic screen of N-ethyl-N-nitrosourea mutagenized Xenopus tropicalis has identified an inner ear mutant named eclipse (ecl). Mutants developed enlarged otic vesicles and various defects of otoconia development; they also showed abnormal circular and inverted swimming patterns. Positional cloning identified specificity protein 8 (sp8), which was previously found to regulate limb and brain development. Two different loss-of-function approaches using transcription activator-like effector nucleases and morpholino oligonucleotides confirmed that the ecl mutant phenotype is caused by down-regulation of sp8. Depletion of sp8 resulted in otic dysmorphogenesis, such as uncompartmentalized and enlarged otic vesicles, epithelial dilation with abnormal sensory end organs. When overexpressed, sp8 was sufficient to induce ectopic otic vesicles possessing sensory hair cells, neurofilament innervation in a thickened sensory epithelium, and otoconia, all of which are found in the endogenous otic vesicle. We propose that sp8 is an important factor for initiation and elaboration of inner ear development.

PubMed ID: 24722637
PMC ID: PMC4035935
Article link: Proc Natl Acad Sci U S A
Grant support: [+]
Genes referenced: actl6a ctnna1 dkk1 egr2 epha4 fgf8 foxj1.2 gbx2.1 gbx2.2 isl1 maf mafb msx1 neurod1 oc90 otx2 pax2 pax8 pc.1 six1 sox13 sox2 sp8 tbx1 tbx2 tuba4b tubb2b
Antibodies: Ctnna1 Ab2 H3f3a Ab9 Isl1 Ab1 Tuba4b Ab4 Tuba4b Ab5
Morpholinos: sp8 MO1 sp8 MO2


Article Images: [+] show captions
References:
Bell, 2003, Pubmed [+]


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