{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Ozawa Y"],"funding":["Grants-in-Aid for Scientific Research by the Japan Society of the Promotion of Science"],"pagination":["1497"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9099608"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["11(9)"],"pubmed_abstract":["Retinitis pigmentosa (RP) is a hereditary blinding disease characterized by gradual photoreceptor death, which lacks a definitive treatment. Here, we demonstrated the effect of 4-phenylbutyric acid (PBA), a chemical chaperon that can suppress endoplasmic reticulum (ER) stress, in P23H mutant rhodopsin knock-in RP models. In the RP models, constant PBA treatment led to the retention of a greater number of photoreceptors, preserving the inner segment (IS), a mitochondrial- and ER-rich part of the photoreceptors. Electroretinography showed that PBA treatment preserved photoreceptor function. At the early point, ER-associated degradation markers, <i>xbp1s</i>, <i>vcp</i>, and <i>derl1</i>, mitochondrial kinetic-related markers, <i>fis1</i>, <i>lc3</i>, and <i>mfn1</i> and <i>mfn2</i>, as well "],"journal":["Cells"],"pubmed_title":["Effects of Epigenetic Modification of PGC-1α by a Chemical Chaperon on Mitochondria Biogenesis and Visual Function in Retinitis Pigmentosa."],"pmcid":["PMC9099608"],"funding_grant_id":["21K09683"],"pubmed_authors":["Ozawa Y","Okano H","Homma K","Tsubota K","Osada H","Toda E","Nagai N"],"additional_accession":[]},"is_claimable":false,"name":"Effects of Epigenetic Modification of PGC-1α by a Chemical Chaperon on Mitochondria Biogenesis and Visual Function in Retinitis Pigmentosa.","description":"Retinitis pigmentosa (RP) is a hereditary blinding disease characterized by gradual photoreceptor death, which lacks a definitive treatment. Here, we demonstrated the effect of 4-phenylbutyric acid (PBA), a chemical chaperon that can suppress endoplasmic reticulum (ER) stress, in P23H mutant rhodopsin knock-in RP models. In the RP models, constant PBA treatment led to the retention of a greater number of photoreceptors, preserving the inner segment (IS), a mitochondrial- and ER-rich part of the photoreceptors. Electroretinography showed that PBA treatment preserved photoreceptor function. At the early point, ER-associated degradation markers, <i>xbp1s</i>, <i>vcp</i>, and <i>derl1</i>, mitochondrial kinetic-related markers, <i>fis1</i>, <i>lc3</i>, and <i>mfn1</i> and <i>mfn2</i>, as well ","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Apr","modification":"2025-04-25T21:32:54.17Z","creation":"2025-02-19T00:55:28.329Z"},"accession":"S-EPMC9099608","cross_references":{"pubmed":["35563803"],"doi":["10.3390/cells11091497"]}}