ArticleBiochimica et biophysica acta2016
Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects.
Article in Biochimica et biophysica acta, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 35 citations in OpenAlex.
- Accelerating Leigh syndrome drug discovery through deep learning screening in brain organoids.Nature communications · 2026Article
- Pluripotent stem-cell-based screening uncovers sildenafil as a mitochondrial disease therapy.Cell · 2026Article
- Mitochondrial translation is the primary determinant of secondary mitochondrial complex I deficiencies.iScience · 2024Article
- Mendelian randomization combined with multi-omics explores the relationship between heart failure and cancer.Journal of Cancer · 2024Article
- Structural rather than catalytic role for mitochondrial respiratory chain supercomplexes.eLife · 2023Article
- Human Tim8a, Tim8b and Tim13 are auxiliary assembly factors of mature Complex IV.EMBO reports · 2023Article
- Dynamic Distribution of HIG2A between the Mitochondria and the Nucleus in Response to Hypoxia and Oxidative Stress.International journal of molecular sciences · 2021Article
- Adeno-associated viral vector serotype 9-based gene replacement therapy forMolecular therapy. Methods & clinical development · 2021Article
- Modelling Mitochondrial Disease in Human Pluripotent Stem Cells: What Have We Learned?International journal of molecular sciences · 2021Review
- Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome.Nature communications · 2021Article
- Review
- Human diseases associated with defects in assembly of OXPHOS complexes.Essays in biochemistry · 2018Review
- SURF1 knockout cloned pigs: Early onset of a severe lethal phenotype.Biochimica et biophysica acta. Molecular basis of disease · 2018Article
- The Organization of Mitochondrial Supercomplexes is Modulated by Oxidative Stress In Vivo in Mouse Models of Mitochondrial Encephalopathy.International journal of molecular sciences · 2018Article
- Tissue- and Condition-Specific Isoforms of Mammalian CytochromeOxidative medicine and cellular longevity · 2017Review
- Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect.Data in brief · 2016Article
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Authors and funding
8 authors at 3 institutions in 3 countries.
Funding
Abstract
Mitochondrial protein SURF1 is a specific assembly factor of cytochrome c oxidase (COX), but its function is poorly understood. SURF1 gene mutations cause a severe COX deficiency manifesting as the Leigh syndrome in humans, whereas in mice SURF1(-/-) knockout leads only to a mild COX defect. We used SURF1(-/-) mouse model for detailed analysis of disturbed COX assembly and COX ability to incorporate into respiratory supercomplexes (SCs) in different tissues and fibroblasts. Furthermore, we compared fibroblasts from SURF1(-/-) mouse and SURF1 patients to reveal interspecies differences in kinetics of COX biogenesis using 2D electrophoresis, immunodetection, arrest of mitochondrial proteosynthesis and pulse-chase metabolic labeling. The crucial differences observed are an accumulation of abundant COX1 assembly intermediates, low content of COX monomer and preferential recruitment of COX into I-III2-IVn SCs in SURF1 patient fibroblasts, whereas SURF1(-/-) mouse fibroblasts were characterized by low content of COX1 assembly intermediates and milder decrease in COX monomer, which appeared more stable. This pattern was even less pronounced in SURF1(-/-) mouse liver and brain. Both the control and SURF1(-/-) mice revealed only negligible formation of the I-III2-IVn SCs and marked tissue differences in the contents of COX dimer and III2-IV SCs, also less noticeable in liver and brain than in heart and muscle. Our studies support the view that COX assembly is much more dependent on SURF1 in humans than in mice. We also demonstrate markedly lower ability of mouse COX to form I-III2-IVn supercomplexes, pointing to tissue-specific and species-specific differences in COX biogenesis.
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