title: Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma).
abstract: Olfactory neuroblastoma (ONB) is a rare neuronal malignancy of the olfactory mucosal. Markers used in the diagnosis of ONB do not distinguish ONB from other neuronal tumors or tumors with neuroendocrine features thus making the diagnosis of ONB difficult. Using a modified RT-PCR technique, we show that the human homologue of the Drosophila achaete-scute gene HASH1 is expressed in 6 primary and one metastatic ONB specimens, whereas Olfactory Marker Protein (OMP) is not. Previous studies have shown that HASH1 is expressed in immature olfactory neurons and is required for their development. OMP, whose function is unknown, is expressed exclusively in mature olfactory neurons. Together, these data suggest that ONB is derived from immature olfactory neurons of neuroectodermal origin. Analysis of RNA expression in primary tumor specimens and in an established cell line make this an ideal system to study olfactory growth and differentiation. Furthermore, these studies represent the first molecular genetic analysis of this rare and unusual neuronal tumor.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Jefferson Medical College, Jefferson Cancer Institute, Philadelphia, PA 19107, USA.
authors.forename: M E
authors.initials: ME
authors.lastname: Carney
authors.forename: R C
authors.initials: RC
authors.lastname: O'Reilly
authors.forename: B
authors.initials: B
authors.lastname: Sholevar
authors.forename: O I
authors.initials: OI
authors.lastname: Buiakova
authors.forename: L D
authors.initials: LD
authors.lastname: Lowry
authors.forename: W M
authors.initials: WM
authors.lastname: Keane
authors.forename: F L
authors.initials: FL
authors.lastname: Margolis
authors.forename: J L
authors.initials: JL
authors.lastname: Rothstein
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D001483:Base Sequence; D051792:Basic Helix-Loop-Helix Proteins; D014408:Biomarkers, Tumor / Q000032:analysis; D017931:DNA Primers; D004273:DNA, Neoplasm / Q000096:biosynthesis / Q000302:isolation & purification; D004268:DNA-Binding Proteins / Q000096:biosynthesis / Q000235:genetics*; D018304:Esthesioneuroblastoma, Olfactory / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D005260:Female; D019922:GAP-43 Protein; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D008875:Middle Aged; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000032:analysis / Q000096:biosynthesis / Q000235:genetics; D009669:Nose Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D051842:Olfactory Marker Protein; D016133:Polymerase Chain Reaction; D012333:RNA, Messenger / Q000032:analysis / Q000235:genetics; D014157:Transcription Factors / Q000096:biosynthesis / Q000235:genetics*; D014407:Tumor Cells, Cultured
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D014408:Biomarkers, Tumor; D017931:DNA Primers; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D019922:GAP-43 Protein; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; D051842:Olfactory Marker Protein; D012333:RNA, Messenger; D014157:Transcription Factors; C065500:ASCL1 protein, human; C497597:OMP protein, human