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

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 regula...

Descripción completa

Detalles Bibliográficos
Autores principales: Golda, Adam, Jurecka, Agnieszka, Gajda, Karolina, Tylki-Szymańska, Anna, Lalik, Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2015
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750576/
https://www.ncbi.nlm.nih.gov/pubmed/26937388
http://dx.doi.org/10.1016/j.ymgmr.2015.02.003

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750576/
https://www.ncbi.nlm.nih.gov/pubmed/26937388
http://dx.doi.org/10.1016/j.ymgmr.2015.02.003

Ejemplares similares