ArticleJournal of cellular and molecular medicine2026
OMICS Profiling Identifies Signatures of Senescence in Osteogenesis Imperfecta Osteoblasts Counteracted by 4-PBA.
Article in Journal of cellular and molecular medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Mutations in collagen I are the most common cause of osteogenesis imperfecta (OI), leading to delayed protein folding and structurally abnormal molecules. While some aberrant collagen is secreted into the extracellular matrix (ECM), impairing bone quality, a significant fraction is retained intracellularly, disrupting osteoblast homeostasis. 4-phenylbutyrate (4-PBA) has been shown to improve osteoblast function and ECM composition in OI models. To investigate the intracellular consequences of mutant collagen retention and the mechanisms of 4-PBA, we analysed the secretome and transcriptome of two dominant OI mouse models, Col1a1
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