title: Math1 is essential for genesis of cerebellar granule neurons.
abstract: The cerebellum is essential for fine motor control of movement and posture, and its dysfunction disrupts balance and impairs control of speech, limb and eye movements. The developing cerebellum consists mainly of three types of neuronal cells: granule cells in the external germinal layer, Purkinje cells, and neurons of the deep nuclei. The molecular mechanisms that underlie the specific determination and the differentiation of each of these neuronal subtypes are unknown. Math1, the mouse homologue of the Drosophila gene atonal, encodes a basic helix-loop-helix transcription factor that is specifically expressed in the precursors of the external germinal layer and their derivatives. Here we report that mice lacking Math1 fail to form granule cells and are born with a cerebellum that is devoid of an external germinal layer. To our knowledge, Math1 is the first gene to be shown to be required in vivo for the genesis of granule cells, and hence the predominant neuronal population in the cerebellum.
authors.affiliation: Department of Molecular and Human Genetics, Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030, USA.
authors.forename: N
authors.initials: N
authors.lastname: Ben-Arie
authors.forename: H J
authors.initials: HJ
authors.lastname: Bellen
authors.forename: D L
authors.initials: DL
authors.lastname: Armstrong
authors.forename: A E
authors.initials: AE
authors.lastname: McCall
authors.forename: P R
authors.initials: PR
authors.lastname: Gordadze
authors.forename: Q
authors.initials: Q
authors.lastname: Guo
authors.forename: M M
authors.initials: MM
authors.lastname: Matzuk
authors.forename: H Y
authors.initials: HY
authors.lastname: Zoghbi
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins; D002454:Cell Differentiation / Q000502:physiology; D002531:Cerebellum / Q000002:abnormalities / Q000166:cytology / Q000196:embryology*; D017353:Gene Deletion; D018390:Gene Targeting; D018257:Helix-Loop-Helix Motifs; D051379:Mice; D009419:Nerve Tissue Proteins / Q000502:physiology*; D009474:Neurons / Q000166:cytology*; D012119:Respiration / Q000502:physiology; D014157:Transcription Factors / Q000502:physiology*
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D009419:Nerve Tissue Proteins; D014157:Transcription Factors; C500943:Atoh1 protein, mouse