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Article

Modeling ALS with iPSCs reveals that mutant SOD1 misregulates neurofilament balance in motor neurons.

Cell stem cell - 5 Jun 2014

Chen Hong, Qian Kun, Du Zhongwei, Cao Jingyuan, Petersen Andrew, Liu Huisheng, Blackbourn Lisle W, Huang CindyTzu-Ling, Errigo Anthony, Yin Yingnan, Lu Jianfeng, Ayala Melvin, Zhang Su-Chun

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) presents motoneuron (MN)-selective protein inclusions and axonal degeneration but the underlying mechanisms of such are unknown. Using induced pluripotent cells (iPSCs) from patients with mutation in the Cu/Zn superoxide dismutase (SOD1) gene, we show that spinal MNs, but rarely non-MNs, exhibited neurofilament (NF) aggregation followed by neurite degeneration when glia were...

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Identifiers

PMID: 24704493
DOI: 10.1016/j.stem.2014.02.004
PMCID: PMC4230530

Genes

SOD1

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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