Human pulmonary artery endothelial cells in the model of mucopolysaccharidosis VI present a prohypertensive phenotype.

Journal: Molecular genetics and metabolism reports
Published Date: Feb 28, 2015

Abstract

BACKGROUND: Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal disorder caused by a deficient activity of N-acetylgalactosamine-4-sulfatase (ARSB). Pulmonary hypertension (PH) occurs in MPS VI patients and is a marker of bad prognosis. Malfunction of endothelium, which regulates vascular tonus and stimulates angiogenesis, can contribute to the occurrence of PH in MPS VI.

Authors

  • Adam Golda
    Department of Cardiology, Gliwice Medical Center, Gliwice, Poland.
  • Agnieszka Jurecka
    Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.
  • Karolina Gajda
    Systems Engineering Group, Faculty of Automatic Control, Electronics and Informatics, Silesian University of Technology, Gliwice, Poland.
  • Anna Tylki-Szymańska
    Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.
  • Anna Lalik
    Systems Engineering Group, Faculty of Automatic Control, Electronics and Informatics, Silesian University of Technology, Gliwice, Poland.

Keywords

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