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CD63-Alix-Rab27a exosome axis is identically influenced in Chediak-Higashi syndrome

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Abstract

Chediak-Higashi syndrome is characterized by congenital abnormality in exocytosis activity, and accumulation of intracellular lysosome-like structures different types of cells within the body. Accordingly, the lack of appropriate exocytosis and deficiency in lysosomal trafficking accounts for immune-related hypoactivity. Exosomes are other types of cell-derived vesicles that play key role in cell-cell cross talk. However, the possible effect of LYST gene mutation in exosome trafficking and biogenesis remains to be clarified. Herein, we investigated the expression of CD63-Alix-Rab27a exosome axis in Chediak-Higashi syndrome. Real-time PCR analysis showed a marked reduction in the expression of CD63, Alix, and Rab27a genes in mononuclear and polymorphonuclear cells. In addition to accumulated number of intracellular lysosome, it seems that the biogenesis and trafficking of exosomes were also abolished.

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Acknowledgments

This study was supported by a grant from Tabriz University of Medical Sciences.

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Authors and Affiliations

  1. Department of Biochemistry and Clinical Laboratories, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran

    Mehdi Hassanpour

  2. Department of Biology, Faculty of Natural Sciences, University of Tabriz, Tabriz, Iran

    Omid Cheraghi

  3. Stem Cell Research Center, Tabriz University of Medical Sciences, Imam Reza St., Golgasht St., Tabriz, 5166614756, Iran

    Maryam Akbarzadeh, Mohammad Nouri & Reza Rahbarghazi

  4. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran

    Saeedeh Zorofchi

  5. Department of Applied Cell Sciences, Faculty of Advanced Medical Sciences, Tabriz University of Medical Sciences, Imam Reza St., Daneshgah St., Tabriz, 5166614756, Iran

    Reza Rahbarghazi

Authors
  1. Mehdi Hassanpour
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  2. Omid Cheraghi
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  3. Maryam Akbarzadeh
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  4. Saeedeh Zorofchi
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  5. Mohammad Nouri
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  6. Reza Rahbarghazi
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Corresponding authors

Correspondence to Mohammad Nouri or Reza Rahbarghazi.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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The authors declare that they have no conflict of interest.

Additional information

Mohammad Nouri and Reza Rahbarghazi contributed equally to this work.

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Hassanpour, M., Cheraghi, O., Akbarzadeh, M. et al. CD63-Alix-Rab27a exosome axis is identically influenced in Chediak-Higashi syndrome. Comp Clin Pathol 25, 1313–1316 (2016). https://doi.org/10.1007/s00580-016-2338-6

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  • DOI: https://doi.org/10.1007/s00580-016-2338-6

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