Ferro-Novick Lab

University of California, San Diego

OTHER BASIC SCIENCES

La Jolla · United States

NIH-funded
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NIH R01 · 2024

The connection between ER-phagy, ER structure and hereditary spastic paraplegias

Project Summary The degradation of misfolded proteins in the endoplasmic reticulum (ER) by the ER-associated degradation (ERAD) pathway prevents potentially toxic proteins from entering the secretory pathway. ERAD, however, cannot clear all proteins from the ER. For example, some proteins, such as aggregation-prone proteins, large polymers and fibrillar proteins, are resistant to degradation by ERAD and must be disposed of by alternate disposal pathways. As aggregation prone proteins have been to linked to neurodegenerative diseases, understanding how these alternate disposal pathways function is of medical importance. ER autophagy (ER-phagy) is a disposal pathway that degrades ER domains…

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