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Article

Proteostasis as a therapeutic target in glomerular injury associated with mutant α-actinin-4.

American journal of physiology. Renal physiology - 1 Oct 2018

Yee Albert, Papillon Joan, Guillemette Julie, Kaufman Daniel R, Kennedy Chris R J, Cybulsky Andrey V

Abstract excerpt

Mutations in α-actinin-4 (actinin-4) result in hereditary focal segmental glomerulosclerosis (FSGS) in humans. Actinin-4 mutants induce podocyte injury because of dysregulation of the cytoskeleton and proteotoxicity. Injury may be associated with endoplasmic reticulum (ER) stress and polyubiquitination of proteins. We assessed if the chemical chaperone 4-phenylbutyrate (4-PBA) can ameliorate the proteotoxicity of...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 29873512
DOI: 10.1152/ajprenal.00082.2018

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