Table 1.

Diseases and defective genes.

Pathway	Gene	Model/disease	Phenotype	Ref.
Protein folding	GRP78	Cardiomyopathy, human	Upregulated	[21]
	GRP78	Myocardial infarction, mouse	Upregulated	[10]
	GRP78	Simulated ischemia, NRVM	Upregulated	[22]
	GRP78	Preconditioning, rat	Upregulated. Knockdown in NRVM dampens protection by preconditioning	[23]
	GRP94	Knockout, mouse	Embryonic lethality; deficient ES cells do not different into cardiac cells	[39–40]
	Calreticulin	Knockout, mouse	Embryonic lethality; ventricular well thinning	[42]
PERK	CHOP	Knockout, mouse	Reduced retinal ganglion cell death after retinal I/R	[46]
	CHOP	Knockout, mouse	Reduced infarction in cardiac I/R	[17]
	CHOP	Atherosclerosis, human	Upregulated in unstable thin-cap atheroma	[47]
	CHOP	Knockout, mouse	Reduced proximal tubule damage in renal I/R	[48]
	CHOP	Cardiomyopathy, human	Upregulated	[50–52]
	CHOP	Knockout, mouse	Reduced pathological remodeling in pressure overload	[50]
IRE	XBP1s	Cardiomyopathy, human	Reduced after implantation of left ventricular assist device	[58]
	XBP1s	Cardiac-specific knockout, mouse	Elevated infarction in cardiac I/R	[58]
	XBP1s	Cardiac-specific overexpression, mouse	Reduced infarction in cardiac I/R	[58]
	XBP1s	Cardiac-specific overexpression, mouse	Reduced infarction in cerebral I/R	[59]
	IRE1	Atherosclerosis, mouse	Inhibitor reduces plaque size	[65]
ATF6	ATF6	Simulated ischemia, NRVM	Upregulated	[68]
	ATF6	Cardiac-specific overexpression, mouse	Reduced infarction in ex vivo cardiac I/R	[69]
	ATF6	Cardiac-specific knockout, mouse	Elevated infarction in cardiac I/R	[70]
	ATF6	Cardiac-specific overexpression, dominant negative mutant	Exacerbated response in mouse myocardial infarction	[71]
	ATF6	ATF6 inhibitor, mouse	Exacerbated response in myocardial infarction	[71]