title: Cilia-lacking respiratory cells in ciliary aplasia.
abstract: This report describes the ultrastructural alterations observed in the nasal and bronchial mucosa of an 11-yr-old male suffering from immotile cilia syndrome (ICS). The morphological features observed in this patient are consistent with a ciliary aplasia. In fact, ciliated cells appeared to be replaced by columnar cells lacking cilia and basal bodies, and bearing on their surface cilium-like projections without any internal axonemal structure. In spite of the absence of basal bodies, centrioles, and kinocilia, these cells unexpectedly showed mature striated roots and centriolar precursor material scattered throughout the apical cytoplasm. These data suggest that control over basal body assembly is distinct from control over striated root formation. The presence of the above-reported structures in cells otherwise presenting many morphological features of normal ciliated cells is discussed on the basis of current knowledge of respiratory cilia biogenesis.
authors.affiliation: Istituti di Anatomia Patologica, Università di Siena, Italy.
authors.forename: M M
authors.initials: MM
authors.lastname: de Santi
authors.forename: C
authors.initials: C
authors.lastname: Gardi
authors.forename: G
authors.initials: G
authors.lastname: Barlocco
authors.forename: M
authors.initials: M
authors.lastname: Canciani
authors.forename: G
authors.initials: G
authors.lastname: Mastella
authors.forename: G
authors.initials: G
authors.lastname: Lungarella
mesh_terms: D001980:Bronchi / Q000002:abnormalities / Q000166:cytology / Q000648:ultrastructure; D002648:Child; D002923:Cilia / Q000473:pathology* / Q000648:ultrastructure; D002925:Ciliary Motility Disorders / Q000473:pathology; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009297:Nasal Mucosa / Q000002:abnormalities / Q000166:cytology / Q000648:ultrastructure; D012137:Respiratory System / Q000166:cytology* / Q000648:ultrastructure; D015619:Respiratory System Abnormalities