ArticleAutophagy2018
Autosomal dominant retinitis pigmentosa-associated gene PRPF8 is essential for hypoxia-induced mitophagy through regulating ULK1 mRNA splicing.
Article in Autophagy, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 51 citations in OpenAlex.
- MicroRNAs as potential prognostic biomarkers in acute lymphoblastic leukemia: a systematic review, meta-analysis, and bioinformatics study.Systematic reviews · 2026Pooled it
- Npl3 is required for efficient autophagosome-vacuole fusion.Autophagy · 2026Article
- Autophagy in the liver.Autophagy reports · 2026Review
- Integrated multi-omics of mitophagy-related molecular subtype characterization and biomarker identification in sepsis.Scientific reports · 2025Article
- Alternative Splicing Dysregulation in Retinitis Pigmentosa: Pathogenic Mechanisms and Therapeutic Opportunities.Biomolecules · 2025Review
- Molecular dynamics of the host response to Streptococcus pneumoniae pneumonia in baboons.Animal models and experimental medicine · 2025Article
- The effect of prolonged G-quadruplex stabilization on the functions of human cells.Scientific reports · 2025Article
- Transcriptomic Alterations in Spliceosome Components in Advanced Heart Failure: Status of Cardiac-Specific Alternative Splicing Factors.International journal of molecular sciences · 2024Article
- The mechanisms of natural products for eye disorders by targeting mitochondrial dysfunction.Frontiers in pharmacology · 2024Review
- Lysosomes in retinal health and disease.Trends in neurosciences · 2023Review
- In vitro and in vivo effects of 3-indoleacetonitrile-A potential new broad-spectrum therapeutic agent for SARS-CoV-2 infection.Antiviral research · 2023Article
- Advances in epigenetic modifications of autophagic process in pulmonary hypertension.Frontiers in immunology · 2023Review
- BNIP3 phosphorylation by JNK1/2 promotes mitophagy via enhancing its stability under hypoxia.Cell death & disease · 2022Article
- Autophagy and beyond: Unraveling the complexity of UNC-51-like kinase 1 (ULK1) from biological functions to therapeutic implications.Acta pharmaceutica Sinica. B · 2022Review
- Autophagy regulation by RNA alternative splicing and implications in human diseases.Nature communications · 2022Review
- Kansl1 haploinsufficiency impairs autophagosome-lysosome fusion and links autophagic dysfunction with Koolen-de Vries syndrome in mice.Nature communications · 2022Article
- Identification of key genes for hypertrophic cardiomyopathy using integrated network analysis of differential lncRNA and gene expression.Frontiers in cardiovascular medicine · 2022Article
- CREG1 improves the capacity of the skeletal muscle response to exercise endurance via modulation of mitophagy.Autophagy · 2021Article
- eIF4A3 regulates the TFEB-mediated transcriptional response via GSK3B to control autophagy.Cell death and differentiation · 2021Article
- BRCA2 deficiency reveals that oxidative stress impairs RNaseH1 function to cripple mitochondrial DNA maintenance.Cell reports · 2021Article
Corrections and comments
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Authors and funding
16 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Aged and damaged mitochondria can be selectively degraded by specific autophagic elimination, termed mitophagy. Defects in mitophagy have been increasingly linked to several diseases including neurodegenerative diseases, metabolic diseases and other aging-related diseases. However, the molecular mechanisms of mitophagy are not fully understood. Here, we identify PRPF8 (pre-mRNA processing factor 8), a core component of the spliceosome, as an essential mediator in hypoxia-induced mitophagy from an RNAi screen based on a fluorescent mitophagy reporter, mt-Keima. Knockdown of PRPF8 significantly impairs mitophagosome formation and subsequent mitochondrial clearance through the aberrant mRNA splicing of ULK1, which mediates macroautophagy/autophagy initiation. Importantly, autosomal dominant retinitis pigmentosa (adRP)-associated PRPF8 mutant R2310K is defective in regulating mitophagy. Moreover, knockdown of other adRP-associated splicing factors, including PRPF6, PRPF31 and SNRNP200, also lead to ULK1 mRNA mis-splicing and mitophagy defects. Thus, these findings demonstrate that PRPF8 is essential for mitophagy and suggest that dysregulation of spliceosome-mediated mitophagy may contribute to pathogenesis of retinitis pigmentosa.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.