Glycoprotein folding and quality-control mechanisms in protein-folding diseases
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چکیده
منابع مشابه
Glycoprotein folding and quality-control mechanisms in protein-folding diseases
Biosynthesis of proteins--from translation to folding to export--encompasses a complex set of events that are exquisitely regulated and scrutinized to ensure the functional quality of the end products. Cells have evolved to capitalize on multiple post-translational modifications in addition to primary structure to indicate the folding status of nascent polypeptides to the chaperones and other p...
متن کاملProtein folding mechanisms
Introduction One of the greatest challenges in modern structural biology is to understand how a newly formed polypeptide sequence finds its correct and unique fold. Real progress has been made towards establishing a fundamental and universal mechanism by which protein folding takes place, and such advances have come from the combination of experimental and theoretical techniques. In our laborat...
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For a protein to function appropriately, it must first achieve its proper conformation and location within the crowded environment inside the cell. Multiple chaperone systems are required to fold proteins correctly. In addition, degradation pathways participate by destroying improperly folded proteins. The intricacy of this multisystem process provides many opportunities for error. Furthermore,...
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A substantial fraction of eukaryotic gene products are synthesized by ribosomes attached at the cytosolic face of the endoplasmic reticulum (ER) membrane. These polypeptides enter cotranslationally in the ER lumen, which contains resident molecular chaperones and folding factors that assist their maturation. Native proteins are released from the ER lumen and are transported through the secretor...
متن کاملProtein folding and quality control in the ER.
The endoplasmic reticulum (ER) uses an elaborate surveillance system called the ER quality control (ERQC) system. The ERQC facilitates folding and modification of secretory and membrane proteins and eliminates terminally misfolded polypeptides through ER-associated degradation (ERAD) or autophagic degradation. This mechanism of ER protein surveillance is closely linked to redox and calcium home...
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ژورنال
عنوان ژورنال: Disease Models & Mechanisms
سال: 2014
ISSN: 1754-8411,1754-8403
DOI: 10.1242/dmm.014589