Mitochondrial repair of 8-oxoguanine is deficient in Cockayne syndrome group B

Stevnsner, Tinna; Nyaga, Simon G.; Souza-Pinto, Nadja C. de; Horst, Gijsbertus T. J. van der; Gorgels, Theo G. M. F.; Hogue, Barbara A.; Thorslund, Tina; Bohr, Vilhelm A.

doi:10.1038/sj.onc.1205994

Cited by 98 publications

(90 citation statements)

References 44 publications

(56 reference statements)

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“…We showed a reduced 8-oxoG incision activity in both the mitochondrial extracts of CSB deficient cells and that of CSB knockout mouse liver cells, indicating a potential role for CSB also in the mitochondrial repair of oxidative base damage (Stevnsner et al, 2002). This activity of the CSB protein in mitochondria is specific for the repair of 8-oxoG, since CSB deficient cells have normal levels of uracil, thymine glycol and hypoxanthine incision activities (Stevnsner et al, 2002). Mitochondria possess an independent BER machinery, the components of which are coded by nuclear genes, resembling nuclear BER in the major molecular steps (described above).…”

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 76%

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 80%

“…Fpg is an E. coli glycosylase that excises 8-oxoG and formamidopyrimidine lesions, followed by DNA strand cleavage. We demonstrated that expression of the wild type or ATPase-domain II mutated CSB gene (CSBE646Q) increased the repair rate of Fpg-sensitive sites in mtDNA of CSB deficient cells (Stevnsner et al, 2002). Furthermore, mitochondrial extracts from these ATPase deficient CSB mutant cells stimulated the repair of Fpg-sensitive sites or the incision of 8-oxoG to the same extent as wildtype CSB extracts, indicating that this domain or ATPase activity of the CSB protein is not required for the mitochondrial repair of oxidative base lesions.…”

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 94%

“…The mtOGG1 protein level is low in CSB deficient cells (Stevnsner et al, 2002). We showed a reduced 8-oxoG incision activity in both the mitochondrial extracts of CSB deficient cells and that of CSB knockout mouse liver cells, indicating a potential role for CSB also in the mitochondrial repair of oxidative base damage (Stevnsner et al, 2002). This activity of the CSB protein in mitochondria is specific for the repair of 8-oxoG, since CSB deficient cells have normal levels of uracil, thymine glycol and hypoxanthine incision activities (Stevnsner et al, 2002).…”

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 97%

“…Certain aspects of the clinical features of CSB overlap with the phenotypes associated with mitochondrial dysfunction, including severe neurological deficiencies, dysfunction in skeletal muscle and heart, and premature aging symptoms. Thus, we have previously suggested that CSB deficient cells accumulate mutations in mtDNA and develop mitochondrial dysfunction that contributes to the phenotype and progression of disease in CSB patients (Stevnsner et al, 2002).…”

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 99%

See 4 more Smart Citations

The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging

Stevnsner

Müftüoğlu

Aamann

et al. 2008

Mechanisms of Ageing and Development

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123

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Cockayne Syndrome (CS) is a rare human genetic disorder characterized by progressive multisystem degeneration and segmental premature aging. The CS complementation group B (CSB) protein is engaged in transcription coupled and global nucleotide excision repair, base excision repair and general transcription. However, the precise molecular function of the CSB protein is still unclear. In the current review we discuss the involvement of CSB in some of these processes, with focus on the role of CSB in repair of oxidative damage, as deficiencies in the repair of these lesions may be an important aspect of the premature aging phenotype of CS.

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Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 76%

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 80%

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 94%

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 97%

Section: The Role Of Csb In Repair Of Oxidative Dna Damagementioning

confidence: 99%

See 3 more Smart Citations

The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging

Stevnsner

Müftüoğlu

Aamann

et al. 2008

Mechanisms of Ageing and Development

Self Cite

123

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show abstract

Xeroderma Pigmentosum and Related Diseases

Schubert

Emmert

2019

Harper's Textbook of Pediatric Dermatology

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Role of mitochondrial dysfunction in the pathophysiology of DNA repair disorders

Mori

Souza-Pinto

2018

Cell Biology International

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DNA is constantly being damaged, either by endogenous or exogenous genotoxins. In that regard, DNA repair activities are essential for maintaining genomic stability and to life itself. Mutations in genes encoding DNA repair proteins cause severe human syndromes, but DNA repair defects have also been linked to several other diseases, notably to cancer and normal aging. Recently, new evidence has emerged indicating that some DNA repair diseases display mitochondrial and metabolic dysfunction through mechanisms that are yet being uncovered. These results suggest that mitochondria play an import role in the DNA damage response pathways and that damage accumulation may lead to mitochondrial dysfunction via metabolic imbalance and mitophagy impairment. Here we review the recent findings linking mitochondrial impairment and cell death to DNA damage accumulation in the context of DNA repair defects. In addition, the general involvement of DNA damage in cellular dysfunction suggests that these phenomena may be also involved in other human pathologies in which mitochondrial dysfunction and metabolic disruption play causative roles.

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Mitochondrial repair of 8-oxoguanine is deficient in Cockayne syndrome group B

Cited by 98 publications

References 44 publications

The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging

The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging

Xeroderma Pigmentosum and Related Diseases

Role of mitochondrial dysfunction in the pathophysiology of DNA repair disorders

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