Zobrazeno 1 - 10
of 21
pro vyhledávání: '"Philip J Thomas"'
Publikováno v:
PLoS ONE, Vol 11, Iss 5, p e0155430 (2016)
The majority of cystic fibrosis (CF)-causing mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) lead to the misfolding, mistrafficking, and degradation of the mutant protein. Inhibition of degradation does not effectively inc
Externí odkaz:
https://doaj.org/article/65e28d82882d4f6392eb128b463d4e69
Autor:
Anna E Patrick, Philip J Thomas
Publikováno v:
Frontiers in Pharmacology, Vol 3 (2012)
Cystic fibrosis is a lethal genetic disease caused by lack of functional cystic fibrosis transmembrane conductance regulator (CFTR) proteins at the apical surface of secretory epithelia. CFTR is a multidomain protein, containing five domains, and its
Externí odkaz:
https://doaj.org/article/ce723ab95e4d4e8688fc68bf3ceca423
Publikováno v:
PLoS ONE, Vol 7, Iss 4, p e35818 (2012)
Amyotrophic Lateral Sclerosis (ALS) is a late-onset, progressive neurodegenerative disease affecting motor neurons in the brain stem and spinal cord leading to loss of voluntary muscular function and ultimately, death due to respiratory failure. A su
Externí odkaz:
https://doaj.org/article/2c1e6fe80ebf4a7f82be020bdb4e0f02
Autor:
Hanneke Hoelen, Bertrand Kleizen, Andre Schmidt, John Richardson, Paraskevi Charitou, Philip J Thomas, Ineke Braakman
Publikováno v:
PLoS ONE, Vol 5, Iss 11, p e15458 (2010)
In the vast majority of cystic fibrosis (CF) patients, deletion of residue F508 from CFTR is the cause of disease. F508 resides in the first nucleotide binding domain (NBD1) and its absence leads to CFTR misfolding and degradation. We show here that
Externí odkaz:
https://doaj.org/article/5e1dfcecc2654050a612b2a5399a73fe
Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization
Autor:
Emile S. Pinarbasi, Tolga Cağatay, Ho Yee Joyce Fung, Ying C. Li, Yuh Min Chook, Philip J. Thomas
Publikováno v:
Scientific Reports, Vol 8, Iss 1, Pp 1-16 (2018)
Abstract ALS (Amyotrophic Lateral Sclerosis) is a neurodegenerative disease characterized by the redistribution of the RNA binding protein TDP-43 in affected neurons: from predominantly nuclear to aggregated in the cytosol. However, the determinants
Externí odkaz:
https://doaj.org/article/e0bc0cffa6c242cf85832575acd6acd8
Autor:
Philip J. Thomas
Publikováno v:
Zeitschrift der Savigny-Stiftung für Rechtsgeschichte. Romanistische Abteilung. 119:544-547
Autor:
Nikolay, Shcheynikov, Shigeru B H, Ko, Weizhong, Zeng, Joo Young, Choi, Michael R, Dorwart, Philip J, Thomas, Shmuel, Muallem
Publikováno v:
Novartis Foundation symposium. 273
Most epithelia that express CFTR secrete fluid rich in HCO3- and poor in Cl- that is generated by a CFTR-dependent Cl- absorption and HCO3- secretion process that when aberrant leads to human diseases such as cystic fibrosis and congenital chloride d
Publikováno v:
Methods in molecular biology (Clifton, N.J.). 301
The 26S proteasome is composed of the core 20S proteasome in association with the 19S regulatory complex, or PA700. PA700 has multiple activities, including ATPase activity, polyubiquitin-chain binding activity, deubiquitination activity, chaperone-l
Publikováno v:
Methods in molecular biology (Clifton, N.J.). 301
Bulk protein degradation in the cell is catalyzed by the ubiquitin-proteasome system (UPS). At the heart of the UPS is the proteasome, a large multisubunit tightly-regulated protease. The UPS performs key functions in protein quality control by monit