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. Author manuscript; available in PMC: 2012 Dec 1.
Published in final edited form as: Biotechnol Bioeng. 2011 Aug 9;108(12):2777–2793. doi: 10.1002/bit.23282

Table 1.

Relevance of ER stress in human disease states

Disease state Role of ER-Stress and UPR Key proteins Reference
Alzheimer's disease (AD) AD-induced Presenilin 1 regulates BiP. Presenilin 1, PERK, eIF2α, BiP Katayama et al. (1999); Milhavet et al. (2002); Niwa et al. (1999); Terro et al. (2002); Unterberger et al. (2006)
Parkinson's disease (PD) Suppressed ER-stress- induced apoptosis and aggregation of α-synuclein. Parkin,α-synuclein Dawson and Dawson (2003); Imai et al. (2000); Petrucelli et al. (2002); Ryu et al. (2002); Takahashi et al. (2003)
Amyotrophic lateral sclerosis (ALS) Altered ERAD machinery and activation of ASK1. Cu/Zn-superoxide dismutase (SOD), ASK1 and Derlin-1 Nishitoh et al. (2008)
Bipolar Disorder Current medications target induction of UPR. XBP1 Cichon et al. (2004); Kakiuchi et al. (2005, 2003)
Stroke CHOP and UPR mediates neuronal response in Ischemia. PERK-eIF2α, ASK1, CHOP Kumar et al. (2001); Paschen et al. (1998); Tajiri et al. (2004); Tanaka et al. (2000)
Heart Disease Degeneration of cardiac myocytes, transaortic constriction. Myocardial infarction induces UPR. IRE1, PERK- eIF2α, ASK1 Glembotski (2008); Okada et al. (2004); Pan et al. (2004); Shintani-Ishida et al. (2006); Thuerauf et al. (2006); Vitadello et al. (2003)
Atherosclerosis Regulates inflammatory genes in vascular cells. IRE1, JNK, TRAF2, XBP1 Gargalovic et al. (2006); Harding et al. (2001); Lee et al. (2008); Zhang et al. (2001)
Type 1 diabetes Regulators in normal conditions and triggers β cell dysfunction and apoptosis in ER Stress. PERK-eIF2α, JNK and Calcium Araki et al. (2003); Fonseca et al. (2005); Ishihara et al. (2004); Oyadomari et al. (2002, 2001); Scheuner et al. (2001); Yamada et al. (2006)
Type 2 diabetes ER stress is a key aspect of type 2 diabetes. XBP1, JNK Ozcan et al. (2004)
Cancer Cytoprotective branches of UPR vital to survival and progression of tumors. BiP, XBP1, ATF6, PERK Bi et al. (2005); Fujimoto et al. (2003); Jamora et al. (1996); Romero-Ramirez et al. (2004); Shuda et al. (2003)
Autoimmune disease Development of plasma cells and dendritic cells. XBP1, HLA-B27 Blass et al. (2001); Corrigall et al. (2001); Todd et al. (2008)
Glomerulonephritis, acute kidney injury Upregulation of BiP, CHOP and down- regulation of Bcl-2 in primary glomerular diseases. BiP, CHOP, Bcl2 Blass et al. (2001); Corrigall et al. (2001); Todd et al. (2008) Bando et al. (2004); Markan et al. (2009)