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Article

Striatal neurons directly converted from Huntington's disease patient fibroblasts recapitulate age-associated disease phenotypes.

Nature neuroscience - 1 Mar 2018

Victor Matheus B, Richner Michelle, Olsen Hannah E, Lee Seong Won, Monteys Alejandro M, Ma Chunyu, Huh Christine J, Zhang Bo, Davidson Beverly L, Yang X William, Yoo Andrew S

Abstract excerpt

In Huntington's disease (HD), expansion of CAG codons in the huntingtin gene (HTT) leads to the aberrant formation of protein aggregates and the differential degeneration of striatal medium spiny neurons (MSNs). Modeling HD using patient-specific MSNs has been challenging, as neurons differentiated from induced pluripotent stem cells are free of aggregates and lack an overt cell death phenotype. Here we generated...

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Identifiers

PMID: 29403030
DOI: 10.1038/s41593-018-0075-7
PMCID: PMC5857213

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HTT

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026.

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