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Article

Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.

Proceedings of the National Academy of Sciences of the United States of America - 10 Apr 2012

Bilican Bilada, Serio Andrea, Barmada Sami J, Nishimura Agnes Lumi, Sullivan Gareth J, Carrasco Monica, Phatnani Hemali P, Puddifoot Clare A, Story David, Fletcher Judy, Park In-Hyun, Friedman Brad A, Daley George Q, Wyllie David J A, Hardingham Giles E, Wilmut Ian, Finkbeiner Steven, Maniatis Tom, Shaw Christopher E, Chandran Siddharthan

Abstract excerpt

Transactive response DNA-binding (TDP-43) protein is the dominant disease protein in amyotrophic lateral sclerosis (ALS) and a subgroup of frontotemporal lobar degeneration (FTLD-TDP). Identification of mutations in the gene encoding TDP-43 (TARDBP) in familial ALS confirms a mechanistic link between misaccumulation of TDP-43 and neurodegeneration and provides an opportunity to study TDP-43 proteinopathies in...

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Cell Differentiation
Detergents
Fibroblasts
Induced Pluripotent Stem Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Organ Specificity

TDP-43 Proteinopathies

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Identifiers

PMID: 22451909
DOI: 10.1073/pnas.1202922109
PMCID: PMC3326463

Genes

TARDBP

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.PMID 22451909Read ↗

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