4 332

Cited 12 times in

Pax3 function is required specifically for inner ear structures with melanogenic fates

Authors
 HongKyung Kim  ;  Harinarayana Ankamreddy  ;  Dong Jin Lee  ;  Kyoung-Ah Kong  ;  Hyuk Wan Ko  ;  Myoung Hee Kim  ;  Jinwoong Bok 
Citation
 BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, Vol.445(3) : 608-614, 2014 
Journal Title
 BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 
ISSN
 0006-291X 
Issue Date
2014
MeSH
Animals ; Cell Differentiation ; Cell Movement ; Ear, Inner/abnormalities* ; Ear, Inner/embryology ; Ear, Inner/metabolism ; Gene Deletion ; Gene Expression Regulation, Developmental ; Hearing Loss/genetics ; Humans ; Melanocytes/cytology* ; Melanocytes/metabolism ; Mice ; Mutation ; Neural Crest/abnormalities ; Neural Crest/embryology ; Neural Crest/metabolism ; PAX3 Transcription Factor ; Paired Box Transcription Factors/genetics ; Paired Box Transcription Factors/metabolism*
Keywords
Hearing loss ; Inner ear ; Melanocyte ; Neural crest ; Pax3
Abstract
Pax3 mutations result in malformed inner ears in Splotch mutant mice and hearing loss in humans with Waardenburg’s syndrome type I. In the inner ear, Pax3 is thought to be involved mainly in the development of neural crest. However, recent studies have shown that Pax3-expressing cells contribute extensively to multiple inner ear structures, some of which were considered to be derived from the otic epithelium. To examine the specific functions of Pax3 during inner ear development, fate mapping of Pax3 lineage was performed in the presence or absence of functional Pax3 proteins using Pax3Cre knock-in mice bred to Rosa26 reporter (R26R) line. β-gal-positive cells were widely distributed in Pax3Cre/+; R26R inner ears at embryonic day (E) 15.5, including the endolymphatic duct, common crus, cristae, maculae, cochleovestibular ganglion, and stria vascularis. In the absence of Pax3 in Pax3Cre/Cre; R26R inner ears, β-gal-positive cells disappeared from regions with melanocytes such as the stria vascularis of the cochlea and dark cells in the vestibule. Consistently, the expression of Dct, a melanoblast marker, was also absent in the mutant inner ears. However, when examined at E11.5, β-gal positive cells were present in Pax3Cre/Cre mutant otocysts, whereas Dct expression was absent, suggesting that Pax3 lineage with a melanogenic fate migrated to the inner ear, yet failed to differentiate and survive without Pax3 function. Gross inner ear morphology was generally normal in Pax3Cre/Cre mutants, unless neural tube defects extended to the cranial region. Taken together, these results suggest that despite the extensive contribution of Pax3-expressing cells to multiple inner ear tissues, Pax3 function is required specifically for inner ear components with melanogenic fates.
Full Text
http://www.sciencedirect.com/science/article/pii/S0006291X1400309X
DOI
10.1016/j.bbrc.2014.02.047
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Anatomy (해부학교실) > 1. Journal Papers
Yonsei Authors
Kong, Kyoung Ah(공경아)
Kim, Myoung Hee(김명희) ORCID logo https://orcid.org/0000-0001-5652-1452
Kim, Hong Kyung(김홍경)
Bok, Jin Woong(복진웅) ORCID logo https://orcid.org/0000-0003-1958-1872
Ankamreddy, Harinarayana(안캄레디하리) ORCID logo https://orcid.org/0000-0002-2859-087X
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/98448
사서에게 알리기
  feedback

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse

Links