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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Detalles Bibliográficos
Autores principales: Haddock, Christopher J., Blomenkamp, Keith, Gautam, Madhav, James, Jared, Mielcarska, Joanna, Gogol, Edward, Teckman, Jeffrey, Skowyra, Dorota
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4161314/
https://www.ncbi.nlm.nih.gov/pubmed/25210780
http://dx.doi.org/10.1371/journal.pone.0106371

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4161314/
https://www.ncbi.nlm.nih.gov/pubmed/25210780
http://dx.doi.org/10.1371/journal.pone.0106371

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