Concepedia

Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency

A. Ordóñez, Erik L. Snapp, Lu Tan, Elena Miranda, Stefan J. Marciniak, David A. Lomas

Hepatology · 2012 · 133 citations · 26 references

DOIFull text

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Abstract

Changes in protein diffusion provide an explanation for the cellular consequences of ER protein overload in mutants that cause inclusion body formation and α1AT deficiency.

References

26

Control of inositol 1,4,5-trisphosphate-induced Ca2+ release by cytosolic Ca2+

Martin D. Bootman, Ludwig Missiaen, Jan B. Parys et al. · Biochemical Journal · 1995 · 5.1K citations

CHOP induces death by promoting protein synthesis and oxidation in the stressed endoplasmic reticulum

Stefan J. Marciniak, Chi Young Yun, Seiichi Oyadomari et al. · Genes & Development · 2004 · 2K citations · Full text

, , +20

The mechanism of Z α1-antitrypsin accumulation in the liver

David A. Lomas, Dyfed LI-Evans, J.T. Finch et al. · Nature · 1992 · 1K citations

, , +7

Orchestrating the unfolded protein response in health and disease

Randal J. Kaufman · Journal of Clinical Investigation · 2002 · 995 citations · Full text

An Autophagy-Enhancing Drug Promotes Degradation of Mutant α <sub>1</sub> -Antitrypsin Z and Reduces Hepatic Fibrosis

Tunda Hidvegi, Michael Ewing, Pamela Hale et al. · Science · 2010 · 593 citations