Next Article in Journal
Modeling Alzheimer’s and Other Age Related Human Diseases in Embryonic Systems
Next Article in Special Issue
Insights into the Etiology of Mammalian Neural Tube Closure Defects from Developmental, Genetic and Evolutionary Studies
Previous Article in Journal
The Role of Hedgehog Signalling in the Formation of the Ventricular Septum
Previous Article in Special Issue
The α-Tubulin gene TUBA1A in Brain Development: A Key Ingredient in the Neuronal Isotype Blend
Article Menu

Export Article

Open AccessArticle
J. Dev. Biol. 2017, 5(4), 18; https://doi.org/10.3390/jdb5040018

Ttc21b Is Required in Bergmann Glia for Proper Granule Cell Radial Migration

1
Division of Human Genetics, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA
2
Division of Developmental Biology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA
*
Author to whom correspondence should be addressed.
Received: 26 August 2017 / Revised: 30 November 2017 / Accepted: 11 December 2017 / Published: 19 December 2017
(This article belongs to the Special Issue Development of the Brain in Health and Disease)
Full-Text   |   PDF [4079 KB, uploaded 19 December 2017]   |  

Abstract

Proper cerebellar development is dependent on tightly regulated proliferation, migration, and differentiation events. Disruptions in any of these leads to a range of cerebellar phenotypes from ataxia to childhood tumors. Animal models have shown that proper regulation of sonic hedgehog (Shh) signaling is crucial for normal cerebellar architecture, and increased signaling leads to cerebellar tumor formation. Primary cilia are known to be required for the proper regulation of multiple developmental signaling pathways, including Shh. Tetratricopeptide Repeat Domain 21B (Ttc21b) is required for proper primary cilia form and function, and is primarily thought to restrict Shh signaling. Here we investigated a role for Ttc21b in cerebellar development. Surprisingly, Ttc21b ablation in Bergmann glia resulted in the accumulation of ectopic granule cells in the lower/posterior lobes of the cerebellum and a reduction in Shh signaling. Ttc21b ablation in just Purkinje cells resulted in a similar phenotype seen in fewer cells, but across the entire extent of the cerebellum. These results suggest that Ttc21b expression is required for Bergmann glia structure and signaling in the developing cerebellum, and in some contexts, augments rather than attenuates Shh signaling. View Full-Text
Keywords: Ttc21b; cilia; Shh; cerebellum; postnatal; migration; Purkinje cell; Bergmann glia; Cre; LoxP Ttc21b; cilia; Shh; cerebellum; postnatal; migration; Purkinje cell; Bergmann glia; Cre; LoxP
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

Supplementary material

SciFeed

Share & Cite This Article

MDPI and ACS Style

Driver, A.M.; Shumrick, C.; Stottmann, R.W. Ttc21b Is Required in Bergmann Glia for Proper Granule Cell Radial Migration. J. Dev. Biol. 2017, 5, 18.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
J. Dev. Biol. EISSN 2221-3759 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top