The engrailed homeobox genes are required in multiple cell lineages to coordinate sequential formation of fissures and growth of the cerebellum.

Developmental Biology
Grant D OrvisA L Joyner

Abstract

The layered cortex of the cerebellum is folded along the anterior-posterior axis into lobules separated by fissures, allowing the large number of cells needed for advanced cerebellar functions to be packed into a small volume. During development, the cerebellum begins as a smooth ovoid structure with two progenitor zones, the ventricular zone and upper rhombic lip, which give rise to distinct cell types in the mature cerebellum. Initially, the cerebellar primordium is divided into five cardinal lobes, which are subsequently further subdivided by fissures. The cellular processes and genes that regulate the formation of a normal pattern of fissures are poorly understood. The engrailed genes (En1 and En2) are expressed in all cerebellar cell types and are critical for regulating formation of specific fissures. However, the cerebellar cell types that En1 and En2 act in to control growth and/or patterning of fissures has not been determined. We conditionally eliminated En2 or En1 and En2 either in both progenitor zones and their descendents or in the two complementary sets of cells derived from each progenitor zone. En2 was found to be required only transiently in the progenitor zones and their immediate descendents to regulate form...Continue Reading

References

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Aug 6, 2004·Neuron·Mark ZervasAlexandra L Joyner
Mar 31, 2006·Development·JoMichelle D CorralesAlexandra L Joyner
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Jul 30, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Roy V SillitoeAlexandra L Joyner

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Citations

Jun 19, 2013·Orphanet Journal of Rare Diseases·Nadia A AkawiLihadh Al-Gazali
Jun 4, 2015·NeuroImage·Kamila U SzulcDaniel H Turnbull
Jan 28, 2015·Clinical Neuroradiology·S HattapoğluF Ekici
Jul 22, 2014·Seminars in Cell & Developmental Biology·Olivier Stettler, Kenneth L Moya
Jul 26, 2012·PloS One·Jennifer BrielmaierJacqueline N Crawley
Apr 18, 2018·Journal of Neuroscience Research·Edwin A ObanaMartin L Doughty
Nov 24, 2017·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Danielle E WhittakerM Albert Basson
Feb 7, 2017·The Journal of Clinical Investigation·Danielle E WhittakerM Albert Basson
Mar 14, 2018·Proceedings of the National Academy of Sciences of the United States of America·I-Li TanAlexandra L Joyner
Oct 7, 2015·The Cerebellum·Ketty LetoRichard Hawkes

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