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4-phenylbutyric acid and Osteogenesis Imperfecta

4-phenylbutyric acid has been researched along with Osteogenesis Imperfecta in 3 studies

4-phenylbutyric acid: RN refers to the parent cpd
4-phenylbutyric acid : A monocarboxylic acid the structure of which is that of butyric acid substituted with a phenyl group at C-4. It is a histone deacetylase inhibitor that displays anticancer activity. It inhibits cell proliferation, invasion and migration and induces apoptosis in glioma cells. It also inhibits protein isoprenylation, depletes plasma glutamine, increases production of foetal haemoglobin through transcriptional activation of the gamma-globin gene and affects hPPARgamma activation.

Osteogenesis Imperfecta: COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperfect dentin formation. Most types are autosomal dominant and are associated with mutations in COLLAGEN TYPE I.

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's2 (66.67)24.3611
2020's1 (33.33)2.80

Authors

AuthorsStudies
Duangchan, T1
Tawonsawatruk, T1
Angsanuntsukh, C1
Trachoo, O1
Hongeng, S1
Kitiyanant, N1
Supokawej, A1
Besio, R2
Iula, G1
Garibaldi, N2
Cipolla, L2
Sabbioneda, S2
Biggiogera, M2
Marini, JC1
Rossi, A2
Forlino, A2
Leoni, L1
Mottes, M1
Aglan, M1
Otaify, GA1
Temtamy, SA1

Other Studies

3 other studies available for 4-phenylbutyric acid and Osteogenesis Imperfecta

ArticleYear
Amelioration of osteogenesis in iPSC-derived mesenchymal stem cells from osteogenesis imperfecta patients by endoplasmic reticulum stress inhibitor.
    Life sciences, 2021, Aug-01, Volume: 278

    Topics: Apoptosis; Cells, Cultured; Endoplasmic Reticulum Chaperone BiP; Endoplasmic Reticulum Stress; Human

2021
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, 2018, Volume: 1864, Issue:5 Pt A

    Topics: Autophagy; Autophagy-Related Protein 5; Cells, Cultured; Collagen Type I; eIF-2 Kinase; Fibroblasts;

2018
Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate.
    Disease models & mechanisms, 2019, 06-20, Volume: 12, Issue:6

    Topics: Apoptosis; Autophagy; Cell Survival; Cells, Cultured; Collagen; Fibroblasts; Homeostasis; Humans; Hy

2019