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Oxidative DNA Damage and Activation of c-Jun N-Terminal Kinase Pathway in Fibroblasts from Patients with Hereditary Spastic Paraplegia

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Summary

  1. 1.

    Hereditary spastic paraplegia (HSP) is a genetically heterogeneous group of neurodegenerative disorders affecting 1 in 10,000 individuals. The present study was aimed to elucidate the role played by reactive oxygen species (ROS) in the pathogenesis of this disease.

  2. 2.

    To address this question we used 7–11 passaged fibroblasts from HSP patients to measure the extent of DNA damage induced by H2O2 treatment and to evaluate the JNK phosphorylation level after hydrogen peroxide and serum stimuli.

  3. 3.

    The present study demonstrates that HSP cells compared to controls are more sensitive to DNA damages induced by H2O2 treatment, and that JNK phosphorylation levels are increased in HSP fibroblasts compared to controls after hydrogen peroxide and serum stimuli. These results suggest a ROS-mediated pathogenetic mechanism for this disease.

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Authors and Affiliations

  1. Dipartimento di Biologia e Patologia Cellulare e Molecolare, Università degli Studi di Napoli “Federico II, Naples, Italy

    Andreina Milano, Raffaele Teperino & Sergio Cocozza

  2. Dipartimento di Biochimica e Biotecnologie Mediche, Università degli Studi di Napoli “Federico II, Naples, Italy

    Nicola Montesano Gesualdi & Franca Esposito

  3. Istituto di Endocrinologia ed Oncologia Sperimentale “G. Salvatore,” Consiglio Nazionale delle Ricerche, Via S. Pansini, Naples, 5-80131, Italy

    Paola Ungaro

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  1. Andreina Milano
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  2. Nicola Montesano Gesualdi
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  3. Raffaele Teperino
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  4. Franca Esposito
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  5. Sergio Cocozza
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  6. Paola Ungaro
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Correspondence to Paola Ungaro.

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Milano, A., Gesualdi, N.M., Teperino, R. et al. Oxidative DNA Damage and Activation of c-Jun N-Terminal Kinase Pathway in Fibroblasts from Patients with Hereditary Spastic Paraplegia. Cell Mol Neurobiol 25, 1245–1254 (2005). https://doi.org/10.1007/s10571-005-8501-2

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