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Article

Analysis of proteome-wide degradation dynamics in ALS SOD1 iPSC-derived patient neurons reveals disrupted VCP homeostasis.

Cell reports - 31 Oct 2023

Tsioras Konstantinos, Smith Kevin C, Edassery Seby L, Garjani Mehraveh, Li Yichen, Williams Chloe, McKenna Elizabeth D, Guo Wenxuan, Wilen Anika P, Hark Timothy J, Marklund Stefan L, Ostrow Lyle W, Gilthorpe Jonathan D, Ichida Justin K, Kalb Robert G, Savas Jeffrey N, Kiskinis Evangelos

Abstract excerpt

Mutations in SOD1 cause amyotrophic lateral sclerosis (ALS) through gain-of-function effects, yet the mechanisms by which misfolded mutant SOD1 (mutSOD1) protein impairs human motor neurons (MNs) remain unclear. Here, we use induced-pluripotent-stem-cell-derived MNs coupled to metabolic stable isotope labeling and mass spectrometry to investigate proteome-wide degradation dynamics. We find several proteins,...

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Identifiers

PMID: 37776851
DOI: 10.1016/j.celrep.2023.113160
PMCID: PMC10785776

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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