Glucose transporter type 10—lacking in arterial tortuosity syndrome—facilitates dehydroascorbic acid transport

Csilla E. Németh, Paola Marcolongo, Alessandra Gamberucci, Rosella Fulceri, Angiolo Benedetti, Nicoletta Zoppi, Marco Ritelli, Nicola Chiarelli, Marina Colombi, Andy Willaert, Bert L. Callewaert, Paul J. Coucke, Pál Gróf, Szilvia K. Nagy, Tamás Mészáros, Gábor Bánhegyi, Éva Margittai

Research output: Contribution to journalLetter

12 Citations (Scopus)

Abstract

Loss-of-function mutations in the gene encoding GLUT10 are responsible for arterial tortuosity syndrome (ATS), a rare connective tissue disorder. In this study GLUT10-mediated dehydroascorbic acid (DAA) transport was investigated, supposing its involvement in the pathomechanism. GLUT10 protein produced by in vitro translation and incorporated into liposomes efficiently transported DAA. Silencing of GLUT10 decreased DAA transport in immortalized human fibroblasts whose plasma membrane was selectively permeabilized. Similarly, the transport of DAA through endomembranes was markedly reduced in fibroblasts from ATS patients. Re-expression of GLUT10 in patients’ fibroblasts restored DAA transport activity. The present results demonstrate that GLUT10 is a DAA transporter and DAA transport is diminished in the endomembranes of fibroblasts from ATS patients.

Original languageEnglish
Pages (from-to)1630-1640
Number of pages11
JournalFEBS letters
DOIs
Publication statusPublished - Jun 1 2016

Keywords

  • Fe/2-oxoglutarate-dependent dehydrogenases
  • GLUT10
  • arterial tortuosity syndrome
  • ascorbate
  • dehydroascorbic acid
  • endomembranes

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Cell Biology

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  • Cite this

    Németh, C. E., Marcolongo, P., Gamberucci, A., Fulceri, R., Benedetti, A., Zoppi, N., Ritelli, M., Chiarelli, N., Colombi, M., Willaert, A., Callewaert, B. L., Coucke, P. J., Gróf, P., Nagy, S. K., Mészáros, T., Bánhegyi, G., & Margittai, É. (2016). Glucose transporter type 10—lacking in arterial tortuosity syndrome—facilitates dehydroascorbic acid transport. FEBS letters, 1630-1640. https://doi.org/10.1002/1873-3468.12204