English
 
User Manual Privacy Policy Disclaimer Contact us
  Advanced SearchBrowse

Item

ITEM ACTIONSEXPORT
  Sp6 and Sp8 transcription factors control AER formation and dorsal-ventral patterning in limb development.

Haro, E., Delgado, I., Junco, M., Yamada, Y., Mansouri, A., Oberg, K. C., et al. (2014). Sp6 and Sp8 transcription factors control AER formation and dorsal-ventral patterning in limb development. PLoS Genetics, 10(8): e1004468. doi:10.1371/journal.pgen.1004468.

Item is

Basic

show hide
Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0023-EC0D-F Version Permalink: http://hdl.handle.net/11858/00-001M-0000-0027-CC85-B
Genre: Journal Article

Files

show Files
hide Files
:
2060624.pdf (Publisher version), 7MB
Name:
2060624.pdf
Description:
-
Visibility:
Public
MIME-Type / Checksum:
application/pdf / [MD5]
Technical Metadata:
Copyright Date:
-
Copyright Info:
-
License:
-
:
2060624_Suppl.zip (Supplementary material), 10MB
Name:
2060624_Suppl.zip
Description:
-
Visibility:
Public
MIME-Type / Checksum:
application/zip / [MD5]
Technical Metadata:
Copyright Date:
-
Copyright Info:
-
License:
-

Creators

show
hide
 Creators:
Haro, E., Author
Delgado, I., Author
Junco, M., Author
Yamada, Y., Author
Mansouri, A.1, Author              
Oberg, K. C., Author
Ros, M. A., Author
Affiliations:
1Research Group of Molecular Cell Differentiation, MPI for biophysical chemistry, Max Planck Society, ou_578588              

Content

show
hide
Free keywords: -
 Abstract: The formation and maintenance of the apical ectodermal ridge (AER) is critical for the outgrowth and patterning of the vertebrate limb. The induction of the AER is a complex process that relies on integrated interactions among the Fgf, Wnt, and Bmp signaling pathways that operate within the ectoderm and between the ectoderm and the mesoderm of the early limb bud. The transcription factors Sp6 and Sp8 are expressed in the limb ectoderm and AER during limb development. Sp6 mutant mice display a mild syndactyly phenotype while Sp8 mutants exhibit severe limb truncations. Both mutants show defects in AER maturation and in dorsal-ventral patterning. To gain further insights into the role Sp6 and Sp8 play in limb development, we have produced mice lacking both Sp6 and Sp8 activity in the limb ectoderm. Remarkably, the elimination or significant reduction in Sp6;Sp8 gene dosage leads to tetra-amelia; initial budding occurs, but neither Fgf8 nor En1 are activated. Mutants bearing a single functional allele of Sp8 (Sp6−/−;Sp8+/−) exhibit a split-hand/foot malformation phenotype with double dorsal digit tips probably due to an irregular and immature AER that is not maintained in the center of the bud and on the abnormal expansion of Wnt7a expression to the ventral ectoderm. Our data are compatible with Sp6 and Sp8 working together and in a dose-dependent manner as indispensable mediators of Wnt/βcatenin and Bmp signaling in the limb ectoderm. We suggest that the function of these factors links proximal-distal and dorsal-ventral patterning.

Details

show
hide
Language(s): eng - English
 Dates: 2014-08-28
 Publication Status: Published online
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Method: Peer
 Identifiers: DOI: 10.1371/journal.pgen.1004468
 Degree: -

Event

show

Legal Case

show

Project information

show

Source 1

show
hide
Title: PLoS Genetics
Source Genre: Journal
 Creator(s):
Affiliations:
Publ. Info: -
Pages: 16 Volume / Issue: 10 (8) Sequence Number: e1004468 Start / End Page: - Identifier: -