Quality control in the endoplasmic reticulum protein factory.
The endoplasmic reticulum (ER) is a factory where secretory proteins are manufactured, and where stringent quality-control systems ensure that only correctly folded proteins are sent to their final destinations. The changing needs of the ER factory are monitored by integrated signalling pathways that constantly adjust the levels of folding assistants. ER chaperones and signalling molecules are emerging as drug targets in amyloidoses and other protein-conformational diseases.
Sitia
Roberto
R
Università Vita-Salute San Raffaele Scientific Institute, 20132 Milano, Italy. r.sitia@hsr.it
Braakman
Ineke
I
D004194:Disease; D004220:Disulfides / Q000378:metabolism; D004721:Endoplasmic Reticulum / Q000378:metabolism*; D006801:Humans; D014176:Protein Biosynthesis; D017510:Protein Folding; D011506:Proteins / Q000737:chemistry* / Q000378:metabolism; D015398:Signal Transduction
D004220:Disulfides; D011506:Proteins