title: En1 is necessary for survival of neurons in the ventral nuclei of the lateral lemniscus.
abstract: The ventral nuclei of the lateral lemniscus (VNLL) are part of the central auditory system thought to participate in temporal sound processing. While the timing and location of VNLL neurogenesis have been determined, the genetic factors that regulate VNLL neuron development are unknown. Here, we use genetic fate-mapping techniques to demonstrate that all glycinergic and glycinergic/GABAergic VNLL neurons derive from a cellular lineage that expresses the homeobox transcription factor Engrailed 1 (En1). We also show that En1 deletion does not affect migration or adoption of a neuronal cell fate but does lead to VNLL neuron death during development. Furthermore, En1 deletion blocks expression of the transcription factor FoxP1 in a subset of VNLL neurons. Together, these data identify En1 as a gene important for VNLL neuron development and survival. © 2016 Wiley Periodicals, Inc. Develop Neurobiol 76: 1266-1274, 2016.
authors.affiliation: Richard King Mellon Institute for Pediatric Research, Department of Pediatrics, University of Pittsburgh, Pittsburgh, Pennsylvania, 15224.
authors.forename: Stefanie C
authors.initials: SC
authors.lastname: Altieri
authors.affiliation: Richard King Mellon Institute for Pediatric Research, Department of Pediatrics, University of Pittsburgh, Pittsburgh, Pennsylvania, 15224.
authors.forename: Tianna
authors.initials: T
authors.lastname: Zhao
authors.affiliation: Department of Pediatrics, Case Western Reserve University, Cleveland, Ohio, 44106.
authors.forename: Walid
authors.initials: W
authors.lastname: Jalabi
authors.affiliation: Department of Pediatrics, Case Western Reserve University, Cleveland, Ohio, 44106.
authors.forename: Rita R
authors.initials: RR
authors.lastname: Romito-DiGiacomo
authors.affiliation: Richard King Mellon Institute for Pediatric Research, Department of Pediatrics, University of Pittsburgh, Pittsburgh, Pennsylvania, 15224. stephen.maricich@chp.edu.|Childrens' Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, 15224. stephen.maricich@chp.edu.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Maricich
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D001933:Brain Stem / Q000196:embryology / Q000254:growth & development / Q000502:physiology*; D019070:Cell Lineage / Q000502:physiology*; D002470:Cell Survival; D051858:Forkhead Transcription Factors / Q000378:metabolism; D018398:Homeodomain Proteins / Q000502:physiology*; D051379:Mice; D008822:Mice, Transgenic; D009474:Neurons / Q000502:physiology*; D012097:Repressor Proteins / Q000378:metabolism
chemical_list: C080330:En1 protein, mouse; D051858:Forkhead Transcription Factors; C432917:Foxp1 protein, mouse; D018398:Homeodomain Proteins; D012097:Repressor Proteins
keywords: auditory; brainstem; deafness; ear; hearing