title: Expression studies of osteoglycin/mimecan (OGN) in the cochlea and auditory phenotype of Ogn-deficient mice.
abstract: Genes involved in the hearing process have been identified through both positional cloning efforts following genetic linkage studies of families with heritable deafness and by candidate gene approaches based on known functional properties or inner ear expression. The latter method of gene discovery may employ a tissue- or organ-specific approach. Through characterization of a human fetal cochlear cDNA library, we have identified transcripts that are preferentially and/or highly expressed in the cochlea. High expression in the cochlea may be suggestive of a fundamental role for a transcript in the auditory system. Herein we report the identification and characterization of a transcript from the cochlear cDNA library with abundant cochlear expression and unknown function that was subsequently determined to represent osteoglycin (OGN). Ogn-deficient mice, when analyzed by auditory brainstem response and distortion product otoacoustic emissions, have normal hearing thresholds.
authors.affiliation: Department of Genetics, Harvard Medical School, Boston, MA, USA.
authors.forename: Robin E
authors.initials: RE
authors.lastname: Williamson
authors.forename: Keith N
authors.initials: KN
authors.lastname: Darrow
authors.forename: Anne B S
authors.initials: AB
authors.lastname: Giersch
authors.forename: Barbara L
authors.initials: BL
authors.lastname: Resendes
authors.forename: Mingqian
authors.initials: M
authors.lastname: Huang
authors.forename: Gary W
authors.initials: GW
authors.lastname: Conrad
authors.forename: Zheng-Yi
authors.initials: ZY
authors.lastname: Chen
authors.forename: M Charles
authors.initials: MC
authors.lastname: Liberman
authors.forename: Cynthia C
authors.initials: CC
authors.lastname: Morton
authors.forename: Elena S
authors.initials: ES
authors.lastname: Tasheva
mesh_terms: D000818:Animals; D001309:Auditory Threshold; D003051:Cochlea / Q000502:physiology*; D016057:Evoked Potentials, Auditory, Brain Stem; D015870:Gene Expression; D015723:Gene Library; D006309:Hearing / Q000502:physiology*; D034381:Hearing Loss / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics*; D051379:Mice; D008810:Mice, Inbred C57BL; D008808:Mice, Inbred CBA; D008817:Mice, Mutant Strains; D020411:Oligonucleotide Array Sequence Analysis; D017084:Otoacoustic Emissions, Spontaneous; D010641:Phenotype
chemical_list: D036341:Intercellular Signaling Peptides and Proteins; C063488:OGN protein, human; C512906:Ogn protein, mouse