Molecular insights into cortico-striatal miscommunications in Huntington's disease.
Curr Opin Neurobiol
; 48: 79-89, 2018 02.
Article
in En
| MEDLINE
| ID: mdl-29125980
ABSTRACT
Huntington's disease (HD), a dominantly inherited neurodegenerative disease, is defined by its genetic cause, a CAG-repeat expansion in the HTT gene, its motor and psychiatric symptomology and primary loss of striatal medium spiny neurons (MSNs). However, the molecular mechanisms from genetic lesion to disease phenotype remain largely unclear. Mouse models of HD have been created that exhibit phenotypes partially recapitulating those in the patient, and specifically, cortico-striatal disconnectivity appears to be a shared pathogenic event shared by HD mouse models and patients. Molecular studies have begun to unveil converging molecular and cellular pathogenic mechanisms that may account for cortico-striatal miscommunication in various HD mouse models. Systems biological approaches help to illuminate synaptic molecular networks as a nexus for HD cortio-striatal pathogenesis, and may offer new candidate targets to modify the disease.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Synapses
/
Cerebral Cortex
/
Huntington Disease
/
Corpus Striatum
Limits:
Animals
/
Humans
Language:
En
Journal:
Curr Opin Neurobiol
Journal subject:
BIOLOGIA
/
NEUROLOGIA
Year:
2018
Document type:
Article
Affiliation country:
United States