Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines

Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines

<p>Abstract</p> <p>Background</p> <p>Many genetic diseases are due to defects in protein trafficking where the mutant protein is recognized by the quality control systems, retained in the endoplasmic reticulum (ER), and degraded by the proteasome. In many cases, the mut...

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Bibliographic Details
Main Authors: Sampson Heidi M, Lam Hung, Chen Pei-Chun, Zhang Donglei, Mottillo Cristina, Mirza Myriam, Qasim Karim, Shrier Alvin, Shyng Show-Ling, Hanrahan John W, Thomas David Y
Format: Article
Language:English
Published: BMC 2013-01-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Protein trafficking
Corrector
Proteostasis
CFTR
hERG
KCNH2
AVPR2
V2R
ABCC8
SUR1
Online Access:http://www.ojrd.com/content/8/1/11

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