title: Tst-1/Oct-6/SCIP regulates a unique step in peripheral myelination and is required for normal respiration.
abstract: The terminal differentiation of myelinating glia involves complex interactions that culminate in the formation of myelin. The POU domain transcription factor Tst-1/Oct-6/SCIP is expressed transiently during myelination, and we report here that it has a critical role in this developmental process. Deletion of the Tst-1/Oct-6/SCIP gene produces a severe defect in peripheral myelination by arresting Schwann cell maturation before axonal wrapping. Unexpectedly, the activation of major myelin-specific genes appears to be unaffected by the Tst-1/Oct-6/SCIP mutation, demonstrating that multiple, independently regulated events are required for terminal differentiation of Schwann cells. In addition, aberrant differentiation and migration of specific neurons in Tst-1/Oct-6/SCIP mutant homozygotes is associated with a fatal breathing defect, providing a model for investigating the regulation of pulmonary homeostasis.
authors.affiliation: Department of Medicine, University of California, San Diego, La Jolla, 92093-0648, USA.
authors.forename: J R
authors.initials: JR
authors.lastname: Bermingham
authors.forename: S S
authors.initials: SS
authors.lastname: Scherer
authors.forename: S
authors.initials: S
authors.lastname: O'Connell
authors.forename: E
authors.initials: E
authors.lastname: Arroyo
authors.forename: K A
authors.initials: KA
authors.lastname: Kalla
authors.forename: F L
authors.initials: FL
authors.lastname: Powell
authors.forename: M G
authors.initials: MG
authors.lastname: Rosenfeld
mesh_terms: D000818:Animals; D001483:Base Sequence; D002454:Cell Differentiation / Q000235:genetics / Q000502:physiology; D017931:DNA Primers / Q000235:genetics; D005260:Female; D017353:Gene Deletion; D015870:Gene Expression; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D008954:Models, Biological; D008969:Molecular Sequence Data; D009186:Myelin Sheath / Q000502:physiology*; D050816:Octamer Transcription Factor-6; D012119:Respiration / Q000235:genetics / Q000502:physiology*; D012583:Schwann Cells / Q000166:cytology / Q000502:physiology; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology*
chemical_list: D017931:DNA Primers; C494180:Pou3f1 protein, mouse; D014157:Transcription Factors; D050816:Octamer Transcription Factor-6