title: Proneural bHLH genes in development and disease.
abstract: Proneural genes encode evolutionarily conserved basic-helix-loop-helix transcription factors. In Drosophila, proneural genes are required and sufficient to confer a neural identity onto naïve ectodermal cells, inducing delamination and subsequent neuronal differentiation. In vertebrates, proneural genes are expressed in cells that already have a neural identity, but they are still required and sufficient to initiate neurogenesis. In all organisms, proneural genes control neurogenesis by regulating Notch-mediated lateral inhibition and initiating the expression of downstream differentiation genes. The general mode of proneural gene function has thus been elucidated. However, the regulatory mechanisms that spatially and temporally control proneural gene function are only beginning to be deciphered. Understanding how proneural gene function is regulated is essential, as aberrant proneural gene expression has recently been linked to a variety of human diseases-ranging from cancer to neuropsychiatric illnesses and diabetes. Recent insights into proneural gene function in development and disease are highlighted herein.
authors.affiliation: Department of Pediatrics, Alberta Children's Hospital Research Institute, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Carol
authors.initials: C
authors.lastname: Huang
authors.affiliation: Department of Pathology & Laboratory Medicine, Southern Alberta Cancer Research Institute, University of Calgary, Calgary, Alberta, Canada. Electronic address: jawchan@ucalgary.ca.
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Chan
authors.affiliation: Department of Biochemistry and Molecular Biology, Alberta Children's Hospital Research Institute, Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada. Electronic address: cschuurm@ucalgary.ca.
authors.forename: Carol
authors.initials: C
authors.lastname: Schuurmans
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins / Q000235:genetics* / Q000378:metabolism; D002490:Central Nervous System / Q000196:embryology / Q000254:growth & development; D002658:Developmental Disabilities / Q000235:genetics; D003920:Diabetes Mellitus / Q000235:genetics; D029721:Drosophila Proteins / Q000235:genetics / Q000378:metabolism; D007758:Ear, Inner / Q000254:growth & development; D005727:Ganglia, Spinal / Q000166:cytology / Q000502:physiology; D018507:Gene Expression Regulation, Developmental; D006801:Humans; D009369:Neoplasms / Q000235:genetics; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism; D009422:Nervous System Diseases / Q000235:genetics; D009474:Neurons / Q000166:cytology / Q000502:physiology*; D012160:Retina / Q000502:physiology; D014714:Vertebrates / Q000235:genetics
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D029721:Drosophila Proteins; D009419:Nerve Tissue Proteins; C499317:Ascl1 protein, mouse; C500943:Atoh1 protein, mouse; C117638:Atoh7 protein, mouse; C105389:NEUROG1 protein, human; C491183:NEUROG2 protein, human
keywords: Achaete-scute; Ascl1; Atonal; Basic-helix–loop–helix transcription factors; Cancer; Diabetes; Neurog1/2/3; Neuropsychiatric disorders; Peripheral and central nervous systems; Proneural genes