Exploring the zebra finch Taeniopygia guttata as a novel animal model for the speech-language deficit of fragile X syndrome.
Fragile X syndrome (FXS) is the most common cause of inherited intellectual disability and presents with markedly atypical speech-language, likely due to impaired vocal learning. Although current models have been useful for studies of some aspects of FXS, zebra finch is the only tractable lab model for vocal learning. The neural circuits for vocal learning in the zebra finch have clear relationships to the pathways in the human brain that may be affected in FXS. Further, finch vocal learning may be quantified using software designed specifically for this purpose. Knockdown of the zebra finch FMR1 gene may ultimately enable novel tests of therapies that are modality-specific, using drugs or even social strategies, to ameliorate deficits in vocal development and function. In this chapter, we describe the utility of the zebra finch model and present a hypothesis for the role of FMRP in the developing neural circuitry for vocalization.
University of Illinois at Urbana-Champaign, Urbana, IL, USA. cwinogr2@illinois.edu
Claudia
C
Winograd
Stephanie
S
Ceman
D000818:Animals; D004195:Disease Models, Animal; D046369:Finches / Q000033:anatomy & histology / Q000502:physiology*; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000235:genetics / Q000378:metabolism; D005600:Fragile X Syndrome / Q000150:complications* / Q000503:physiopathology*; D055785:Gene Knockdown Techniques; D006801:Humans; D007806:Language Disorders / Q000209:etiology* / Q000503:physiopathology; D007858:Learning / Q000502:physiology; D010641:Phenotype; D013064:Speech Disorders / Q000209:etiology* / Q000503:physiopathology; D014828:Vocalization, Animal / Q000502:physiology*
D051860:Fragile X Messenger Ribonucleoprotein 1