[Huntington's disease: molecular foundations and implications in the characterisation of the neuronal mechanisms responsible for linguistic processing].
BACKGROUND
Certain neuronal models of linguistic processing suggest that the basal ganglia play a key role in this processing, thanks to their integration within the so-called cortico-striato-cortical circuits.


METHODS
A comparative analysis, at a phenotypic and molecular level, of the pathologies, syndromes and disorders that entail a structural alteration and/or a dysfunction of the basal ganglia is essential for validating and optimising such models, as well as for achieving a suitable characterisation of the genetic program responsible for the development and functioning of the 'language organ'. One of the most significant pathologies in this respect is Huntington's disease, which is caused by the destruction of certain groups of neurons in the caudate nucleus.


CONCLUSIONS
This type of analysis seems to confirm the hypothesis that, during linguistic processing, the basal ganglia would be responsible for planning and modulating the sequential tasks related to the so-called procedural (or computational or rule-applying) component of language. Equally plausible, however, is the hypothesis that, inside them, there would be regions that are specifically dedicated to processing the different (morphological and syntactical) rules that go to make up said component. Additionally, the nature of these subcortical structures and the function they perform would explain the simultaneous presence of an articulatory and a linguistic deficit in disorders in which the basal ganglia are affected. Lastly, this kind of analysis is also making it possible to characterise some of the genes that constitute the genetic program responsible for the development and functioning of this region of the brain and, by extension, of the 'language organ'.
Benítez-Burraco
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Departamento de Filología Española, Area de Lingüística, Facultad de Filología, Universidad de Oviedo, Asturias, España. abenitez@us.es
D000818:Animals; D001184:Articulation Disorders / Q000209:etiology / Q000503:physiopathology*; D001479:Basal Ganglia / Q000503:physiopathology*; D001480:Basal Ganglia Diseases / Q000503:physiopathology; D002421:Caudate Nucleus / Q000502:physiology; D019143:Evolution, Molecular; D051858:Forkhead Transcription Factors / Q000235:genetics / Q000502:physiology; D018740:Genetic Heterogeneity; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000503:physiopathology*; D007802:Language; D007806:Language Disorders / Q000209:etiology / Q000503:physiopathology*; D051379:Mice; D008959:Models, Neurological; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000502:physiology; D009474:Neurons / Q000502:physiology*; D009687:Nuclear Proteins / Q000235:genetics / Q000502:physiology; D010641:Phenotype; D000071656:Receptor, Notch3; D018092:Kainic Acid Receptors / Q000235:genetics / Q000502:physiology; D051880:Receptors, Notch / Q000235:genetics / Q000502:physiology; D018911:Trinucleotide Repeats; D016875:tau Proteins / Q000235:genetics / Q000502:physiology; D000097806:GluK2 Kainate Receptor
D051858:Forkhead Transcription Factors; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D000071656:Receptor, Notch3; D018092:Kainic Acid Receptors; D051880:Receptors, Notch; D016875:tau Proteins; D000097806:GluK2 Kainate Receptor; C465448:FOXP2 protein, human; C086055:HTT protein, human; C054369:MAPT protein, human; C499374:NOTCH3 protein, human