Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases

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Adnan, Humaira; Zhang, Zhenbo; Park, Hyun-Joo; Tailor, Chetankumar; Che, Clare; Kamani, Mustafa; Spitalny, George; Binnington, Beth; Lingwood, Clifford;
(2016)
  • Publisher: Public Library of Science
  • Journal: PLoS ONE,volume 11,issue 12 (issn: 1932-6203, eissn: 1932-6203)
  • Publisher copyright policies & self-archiving
  • Related identifiers: pmc: PMC5147855, doi: 10.1371/journal.pone.0166948
  • Subject: Genetic Diseases | Research Article | Inherited Metabolic Disorders | Anatomy | Pathology and Laboratory Medicine | Secretory Pathway | Toxoids | Chemical Compounds | Cell Processes | Fibroblasts | Physical Sciences | Biological Tissue | Connective Tissue | Proteins | Toxicology | Chemistry | Chlorides | Metabolic Disorders | Cellular Types | Biology and Life Sciences | Chaperone Proteins | Epithelial Cells | Medicine | Animal Cells | Connective Tissue Cells | Autosomal Recessive Diseases | Q | R | Cell Biology | Gaucher's Disease | Toxic Agents | Cellular Structures and Organelles | Science | Toxins | Biochemistry | Clinical Genetics | Epithelium | Medicine and Health Sciences | Bacterial Toxins | Endoplasmic Reticulum

Many germ line diseases stem from a relatively minor disturbance in mutant protein endoplasmic reticulum (ER) 3D assembly. Chaperones are recruited which, on failure to correct folding, sort the mutant for retrotranslocation and cytosolic proteasomal degradation (ER-ass... View more
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