Encyclopedia of Biophysics

Living Edition
| Editors: Gordon Roberts, Anthony Watts, European Biophysical Societies

(Glyco)Protein Folding Disorders

  • Elizabeth Hounsell
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-35943-9_102-1

Synonyms

Definition

The term “protein folding disorders” (PFD) was introduced for a small group of proteins that misfold after biosynthesis producing pathological symptoms, for example, by depositing in inappropriate places. Of these proteins, three can be normally or sometimes N-glycosylated hence the term “(glyco)protein folding disorders” (GFD). However one of these, the Aβ precursor glycoprotein providing the 40, 41, or 42 amino acid residue fragments called amyloid-β-protein (Aβ) that are found in the plaques of patients with Alzheimer’s disease (AD), is also O-GlcNAcylated and many other proteins can also be, which results in misfolding and tissue deposition.

Introduction

The term “PFD” can also now be applied to many more proteins, as we find out more about their complicated life stories. For example, in Huntington’s Disease (HD), the addition at gene level of repeat CAG means that the protein can be biosynthesized with a long Gln tail. This is somewhat...

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References

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Copyright information

© European Biophysical Societies' Association (EBSA) 2018

Authors and Affiliations

  1. 1.School of Biological and Chemical SciencesBirkbeck College, University of LondonLondonUK

Section editors and affiliations

  • Elizabeth Hounsell
    • 1
  1. 1.School of Biological and Chemical SciencesBirkbeck College, University of LondonLondonUK