Cargando…
Folliculin haploinsufficiency causes cellular dysfunction of pleural mesothelial cells
Birt–Hogg–Dubé syndrome (BHDS), an autosomal dominant inheritance disease caused by folliculin (FLCN) mutations, is associated with lung cysts and spontaneous pneumothorax. The possibility of FLCN haploinsufficiency in pleural mesothelial cells (PMCs) contributing to development of pneumothorax has...
Autores principales: | Okamoto, Shouichi, Ebana, Hiroki, Kurihara, Masatoshi, Mitani, Keiko, Kobayashi, Etsuko, Hayashi, Takuo, Sekimoto, Yasuhito, Nishino, Koichi, Otsuji, Mizuto, Kumasaka, Toshio, Takahashi, Kazuhisa, Seyama, Kuniaki |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8144428/ https://www.ncbi.nlm.nih.gov/pubmed/34031471 http://dx.doi.org/10.1038/s41598-021-90184-9 |
Ejemplares similares
-
Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
por: Sekimoto, Yasuhito, et al.
Publicado: (2021) -
Haploinsufficiency of the folliculin gene leads to impaired functions of lung fibroblasts in patients with Birt–Hogg–Dubé syndrome
por: Hoshika, Yoshito, et al.
Publicado: (2016) -
A total pleural covering of absorbable cellulose mesh prevents pneumothorax recurrence in patients with Birt-Hogg-Dubé syndrome
por: Mizobuchi, Teruaki, et al.
Publicado: (2018) -
Isolation and characterisation of lymphatic endothelial cells from lung tissues affected by lymphangioleiomyomatosis
por: Nishino, Koichi, et al.
Publicado: (2021) -
Clinical and genetic features of 334 Asian patients with Birt–Hogg–Dubé syndrome (BHDS) who presented with pulmonary cysts with or without a history of pneumothorax, with special reference to BHDS-associated pneumothorax
por: Namba, Yukiko, et al.
Publicado: (2023)