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Increased expression of l-amino acid transporters in balloon cells of tuberous sclerosis

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Abstract

Objects

Tuberous sclerosis complex (TSC) is a dysgenetic syndrome involved in multiple organs, and the pathognomonic cortical tuber act as an epileptic substrate. The amino acid transport system L (LAT) is a major nutrient transport system, and LAT1 is highly expressed in malignant tumors to support tumor cell growth. To study the life-long epilepsy from the cortical tuber, the expression of LAT1 in balloon cells and dysplastic neurons of the cortical tuber is investigated.

Materials and methods

LAT1 expression was investigated by LAT1 mRNA using reverse transcription-polymerase chain reaction and immunohistochemical staining with anti-human LAT1 antibody in nine patients with TSC and three control brains.

Conclusion

LAT1 mRNA was detectable only in fresh-frozen tissues of TSC, and it was upregulated in the cortical tuber lesion. While the LAT1 immunopositivity of control brains was limited in the capillary endothelial cells in the gray matter, increased LAT1 immunopositivity was noted in balloon cells of the cortical tubers in addition to the capillary endothelial cells as shown in control brains. Linear and strong immunopositivity along the cell membrane and cytoplasm of the balloon cells, and weakly granular immunopositivity in their cytoplasm were noted. Increased expression of LAT1 in the balloon cells is important for the active transport of large neutral amino acids into the balloon cells, and that the biologic process may play an important role in the active protein synthesis with metabolic maintenance of balloon cells in cortical tubers of patients with TSC.

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Acknowledgements

This work was partially funded by the Clinical Research Institute, Chonnam National University Hospital (CRI 08051-1).

Disclosure

The authors report no conflict of interest concerning the materials or methods and the results in this study. Author contributions to the study and manuscript preparation include the following. Conception and design: all authors. Acquisition of data: all authors. Analysis and interpretation of data: Lim BC, Kim DK, Lee MC. Drafting the article: Lim BC, Kim HS, Lee KH, Lee MC. Reviewed final version of the manuscript and approved it for submission: all authors.

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Authors and Affiliations

  1. Department of Neurosurgery, Gwangju Christian Hospital, Gwangju, South Korea

    Byung-Chan Lim, Kyu-Young Cho, Joon-Seop Lim & Rae-Seop Lee

  2. Department of Forensic Medicine, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), Gwangju, South Korea

    Hyung-Seok Kim

  3. Department of Neurology, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), Gwangju, South Korea

    Myeong-Kyu Kim

  4. Department of Neurosurgery, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), Gwangju, South Korea

    Jae-Hyoo Kim

  5. Department of Pediatrics, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), Gwangju, South Korea

    Young-Jong Woo

  6. Department of Pharmacology, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), Gwangju, South Korea

    Jong-Keun Kim

  7. Department of Oral Physiology, School of Dentistry, Chosun University, Gwangju, South Korea

    Do Kyung Kim

  8. Department of Bioengineering, Gwangju Institute of Science and Technology, Gwangju, South Korea

    Hyung-Ihl Kim

  9. Department of Pathology, Chonnam National University Medical School and Research Institute of Medical Science, Center for Biomedical Research and Human resources (BK 21), 5 Hakdong, Donggu, Gwangju, 501-746, South Korea

    Kyung-Wha Lee & Min-Cheol Lee

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  1. Byung-Chan Lim
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Correspondence to Min-Cheol Lee.

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Lim, BC., Cho, KY., Lim, JS. et al. Increased expression of l-amino acid transporters in balloon cells of tuberous sclerosis. Childs Nerv Syst 27, 63–70 (2011). https://doi.org/10.1007/s00381-010-1239-2

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  • DOI: https://doi.org/10.1007/s00381-010-1239-2

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