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Article

4-PBA ameliorates cellular homeostasis in fibroblasts from osteogenesis imperfecta patients by enhancing autophagy and stimulating protein secretion.

Biochimica et biophysica acta. Molecular basis of disease - 1 May 2018

Besio Roberta, Iula Giusy, Garibaldi Nadia, Cipolla Lina, Sabbioneda Simone, Biggiogera Marco, Marini Joan C, Rossi Antonio, Forlino Antonella

Abstract excerpt

The clinical phenotype in osteogenesis imperfecta (OI) is attributed to the dominant negative function of mutant type I collagen molecules in the extracellular matrix, by altering its structure and function. Intracellular retention of mutant collagen has also been reported, but its effect on cellular homeostasis is less characterized. Using OI patient fibroblasts carrying mutations in the α1(I) and α2(I) chains...

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Identifiers

PMID: 29432813
DOI: 10.1016/j.bbadis.2018.02.002
PMCID: PMC5908783

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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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