PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes.

PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes.

Accumulation of aggregation-prone human alpha 1 antitrypsin mutant Z (AT-Z) protein in PiZ mouse liver stimulates features of liver injury typical of human alpha 1 antitrypsin type ZZ deficiency, an autosomal recessive genetic disorder. Ubiquitin-mediated proteolysis by the 26S proteasome counteract...

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Bibliographic Details
Main Authors: Christopher J Haddock, Keith Blomenkamp, Madhav Gautam, Jared James, Joanna Mielcarska, Edward Gogol, Jeffrey Teckman, Dorota Skowyra
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4161314?pdf=render

Internet

http://europepmc.org/articles/PMC4161314?pdf=render

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