The Biological and Chemical Basis for Tissue-Selective Amyloid Disease

  title={The Biological and Chemical Basis for Tissue-Selective Amyloid Disease},
  author={Yoshiki Sekijima and R Luke Wiseman and Jeanne L. Matteson and Per Hammarstr{\"o}m and Sean R. Miller and Anu R. Sawkar and William Edward Balch and Jeffery W Kelly},
Factors controlling the onset and progression of extracellular amyloid diseases remain largely unknown. Central to disease etiology is the efficiency of the endoplasmic reticulum (ER) machinery that targets destabilized mutant proteins for degradation and the enhanced tendency of these variants to aggregate if secreted. We demonstrate that mammalian cells secrete numerous transthyretin (TTR) disease-associated variants with wild-type efficiency in spite of compromised folding energetics. Only… CONTINUE READING


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ransthyretin aggregation under partially denaturing conditions is a ownhill polymerization

A. R. urshman, J. T. White, E. T. Powers, J. W. Kelly
Biochemistry • 2004

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