Molecular insights into cortico-striatal miscommunications in Huntington's disease. Curr Opin Neurobiol 2018 Feb;48:79-89
Date
11/11/2017Pubmed ID
29125980Pubmed Central ID
PMC5825262DOI
10.1016/j.conb.2017.10.019Scopus ID
2-s2.0-85032950437 (requires institutional sign-in at Scopus site) 35 CitationsAbstract
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.
Author List
Veldman MB, Yang XWAuthor
Matthew B. Veldman PhD Assistant Professor in the Cell Biology, Neurobiology and Anatomy department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsCerebral Cortex
Corpus Striatum
Humans
Huntington Disease
Synapses
