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Cardiolipin content in mitochondria from cultured skin fibroblasts harboring mutations in the mitochondrial ATP6 gene

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Abstract

The role of phospholipids in normal assembly and organization of the membrane proteins has been well documented. Cardiolipin, a unique tetra-acyl phospholipid localized in the inner mitochondrial membrane, is implicated in the stability of many inner-membrane protein complexes. Loss of cardiolipin content, alterations in its acyl chain composition and/or cardiolipin peroxidation have been associated with dysfunction in multiple tissues in a variety of pathological conditions. The aim of this study was to analyze the phospholipid composition of the mitochondrial membrane in the four most frequent mutations in the ATP6 gene: L156R, L217R, L156P and L217P but, more importantly, to investigate the possible changes in the cardiolipin profile. Mitochondrial membranes from fibroblasts with mutations at codon 217 of the ATP6 gene, showed a different cardiolipin content compared to controls. Conversely, results similar to controls were obtained for mutations at codon 156. These findings may be attributed to differences in the biosynthesis and remodeling of cardiolipin at the level of the inner mitochondrial transmembrane related to some mutations of the ATP6 gene.

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Acknowledgments

This work was supported in part by grants from the Italian Ministry of Health; and by the Second Program of Scientific and Technological Collaboration between Italy and Mexico (Ministero degli Esteri-ITALY and Secretaria de Relaciones Exteriores-Conacyt MEXICO).

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Author notes

  1. Martha Elisa Vázquez-Memije

    Present address: Biochemistry Laboratory, Cedimemm, S.C., Avenida Coyoacán 1120-3, México City, CP 03100, Mexico

Authors and Affiliations

  1. Departamento de Biomedicina Cardiovascular, Instituto Nacional de Cardiología, Juan Badiano No 1 Tlalpan, Mexico, CP 14080, DF, Mexico

    Mohammed El-Hafidi

  2. Unit of Molecular Medicine, Bambino Gesù Children’s Hospital, Rome, Italy

    Maria Chiara Meschini, Teresa Rizza, Enrico Bertini & Rosalba Carrozzo

  3. Molecular Medicine & Neurogenetics, IRCCS Fondazione Stella Maris, Pisa, Italy

    Filippo M. Santorelli

  4. Unidad de Investigación Médica en Genética, Centro Médico Nacional S.XXI. Instituto Mexicano del Seguro Social, Apdo. Postal 73-032, México, DF CP 06725, México

    Martha Elisa Vázquez-Memije

Authors
  1. Mohammed El-Hafidi
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  2. Maria Chiara Meschini
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  3. Teresa Rizza
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  4. Filippo M. Santorelli
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  5. Enrico Bertini
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  6. Rosalba Carrozzo
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  7. Martha Elisa Vázquez-Memije
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Corresponding authors

Correspondence to Rosalba Carrozzo or Martha Elisa Vázquez-Memije.

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El-Hafidi, M., Meschini, M.C., Rizza, T. et al. Cardiolipin content in mitochondria from cultured skin fibroblasts harboring mutations in the mitochondrial ATP6 gene. J Bioenerg Biomembr 43, 683–690 (2011). https://doi.org/10.1007/s10863-011-9387-y

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  • DOI: https://doi.org/10.1007/s10863-011-9387-y

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