The double whammy of ER-retention and dominant-negative effects in numerous autosomal dominant diseases: significance in disease mechanisms and therapy

The double whammy of ER-retention and dominant-negative effects in numerous autosomal dominant diseases: significance in disease mechanisms and therapy

Abstract The endoplasmic reticulum (ER) employs stringent quality control mechanisms to ensure the integrity of protein folding, allowing only properly folded, processed and assembled proteins to exit the ER and reach their functional destinations. Mutant proteins unable to attain their correct tert...

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Bibliographic Details
Main Authors: Nesrin Gariballa, Feda Mohamed, Sally Badawi, Bassam R. Ali
Format: Article
Language:English
Published: BMC 2024-06-01
Series:Journal of Biomedical Science
Subjects:
ERAD
Dominant-negative effects
Misfolded proteins
ER-retention
Heteromeric complexes
Online Access:https://doi.org/10.1186/s12929-024-01054-1

Internet

https://doi.org/10.1186/s12929-024-01054-1

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