The biological and chemical basis for tissue-selective amyloid disease.

Abstract:

: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 the most highly destabilized TTR variants are subjected to ER-associated degradation (ERAD) and then only in certain tissues, providing insight into tissue selective amyloidosis. Rather than a "quality control" standard based on wild-type stability, we find that ER-assisted folding (ERAF), based on global protein energetics, determines the extent of export. We propose that ERAF (influenced by the energetics of the protein fold, chaperone enzyme distributions, and metabolite chaperones) in competition with ERAD defines the unique secretory aptitude of each tissue.

journal_name

Cell

journal_title

Cell

authors

Sekijima Y,Wiseman RL,Matteson J,Hammarström P,Miller SR,Sawkar AR,Balch WE,Kelly JW

doi

10.1016/j.cell.2005.01.018

subject

Has Abstract

pub_date

2005-04-08 00:00:00

pages

73-85

issue

1

eissn

0092-8674

issn

1097-4172

pii

S0092-8674(05)00092-9

journal_volume

121

pub_type

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