The role of surfactant and distal lung dysfunction in the pathology of lysosomal storage diseases

Autor: Paget, Tamara L, Parkinson-Lawrence, Emma J, Orgeig, Sandra

Publikováno v: In Current Opinion in Physiology October 2021 23

Interstitial lung disease and surfactant dysfunction as a secondary manifestation of disease: insights from lysosomal storage disorders

Autor: Paget, Tamara L., Parkinson-Lawrence, Emma J., Orgeig, Sandra ^⁎

Publikováno v: In Drug Discovery Today: Disease Models Summer 2019 29-30:35-42

Contributors

Autor: Abman, Steven H., Albertine, Kurt H., Albertson, Timothy E., Alizani, Gamar, Andino, Ariana L., Bayat, Yasaman, Brown, Anthony P., Capen, Diane E., Cardoso, Wellington V., Cheng, Jiayi, Crowley, Candace, Cutz, Ernest, Davies, Indya M., Dhillon, Jaspreet K., Didier, Elizabeth S., Domnik, Nicolle J., Fisher, John T., Galambos, Csaba, Georgian, Elizabeth, Gershwin, Laurel J., Green, Francis H.Y., Harding, Richard, Hedriana, Herman, Herring, Matt J., Hooper, Stuart B., Hsia, Connie C.W., Hu, Xin, Hyde, Dallas M., Islam, Mahed, Ji, Hong, Jones, Rosemary, Joss-Moore, Lisa A., Kitchen, Marcus J., Kuroda, Marcelo J., Lakshminrusimha, Satyan, Lane, Robert H., Lim, Michelle J., Maritz, Gert S., McCartney, Alexandra C., McDougall, Annie R.A., McGillick, Erin V., Miller, Lisa A., Mori, Munemasa, Morrison, Janna L., Nagle, Jefferson G.C., Noël, Alexandra, O'Reilly, Megan, Orgeig, Sandra, Paget, Tamara L., Parkinson-Lawrence, Emma J., Paulsen, Daniel B., Penn, Arthur L., Pierro, Maria, Pinkerton, Kent E., Poindexter, Morgan E., Reid, Lynne, Saleem, Roma, Schittny, Johannes C., Sozo, Foula, te Pas, Arjan B., Thébaud, Bernard, Wallace, Megan J., Wang, Lei, Westmont, Taylor, Wu, Ching-Wen, Yoder, Bradley A., You, Dorothy, Zablocki-Thomas, Laurent, Zhang, Chuanzhen, Zhu, Tao

Publikováno v: In The Lung Edition: Third Edition. 2025:xv-xvii

Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse

Autor: Sandra Orgeig, Grielof Koster, Marten F. Snel, Mercedes Echaide, Emma J. Parkinson-Lawrence, Paul J. Trim, Chiara Autilio, Tamara L. Paget, Janna L. Morrison, Madhuriben H. Panchal, Jesús Pérez-Gil, Anthony D. Postle

Publikováno v: Cells, Vol 10, Iss 849, p 849 (2021)
E-Prints Complutense. Archivo Institucional de la UCM
instname
E-Prints Complutense: Archivo Institucional de la UCM
Universidad Complutense de Madrid
Cells
Volume 10
Issue 4

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease with significant neurological and skeletal pathologies. Respiratory dysfunction is a secondary pathology contributing to mortality in MPS IIIA patients. Pulmonary surfactant is cruc

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::660afcfd44ba85fe52ddfea5d3ed3256
https://www.mdpi.com/2073-4409/10/4/849

Interstitial lung disease and surfactant dysfunction as a secondary manifestation of disease: insights from lysosomal storage disorders

Autor: Tamara L. Paget, Emma J. Parkinson-Lawrence, Sandra Orgeig

Lysosomal storage disorders are a group of genetic metabolic disorders caused by dysfunctional endosomal-lysosomal hydrolases, altered vesicular trafficking or biogenesis of the lysosome. This results in the accumulation of partially degraded substra

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::985bcb538fa3ac7362636bcf08c9a800
https://hdl.handle.net/11541.2/136758