Endogenous

ERN1 IRE1

Serine/threonine-protein kinase/endoribonuclease IRE1

ERN1 ;
IRE1 ;
Serine/threonine-protein kinase/endoribonuclease IRE1 ;
Endoplasmic reticulum-to-nucleus signaling 1 ;
Inositol-requiring protein 1 ;
hIRE1p ;
Ire1-alpha ;
IRE1a

The protein encoded by this gene is the ER to nucleus signalling 1 protein, a human homologue of the yeast Ire1 gene product. This protein possesses intrinsic kinase activity and an endoribonuclease activity and it is important in altering gene expression as a response to endoplasmic reticulum-based stress signals. [provided by RefSeq, Jul 2008],

Catalytic activity:ATP + a protein = ADP + a phosphoprotein.,cofactor:Magnesium.,enzyme regulation:The kinase domain is activated by trans-autophosphorylation. Kinase activity is required for activation of the endoribonuclease domain.,Function:Senses unfolded proteins in the lumen of the endoplasmic reticulum via its N-terminal domain which leads to enzyme auto-activation. The active endoribonuclease domain splices XBP1 mRNA to generate a new C-terminus, converting it into a potent unfolded-protein response transcriptional activator and triggering growth arrest and apoptosis.,PTM:Autophosphorylated.,similarity:Belongs to the protein kinase superfamily. Ser/Thr protein kinase family.,similarity:Contains 1 KEN domain.,similarity:Contains 1 protein kinase domain.,subunit:Homodimer; disulfide-linked. Dimer formation is driven by hydrophobic interactions within the N-terminal luminal domains and stabilized by disulfide bridges. Also binds HSPA5, a negative regulator of the unfolded protein response. This interaction may disrupt homodimerization and prevent activation of ERN1.,tissue specificity:Ubiquitously expressed. High levels observed in pancreatic tissue.,

Endoplasmic reticulum membrane

Ubiquitously expressed. High levels observed in pancreatic tissue.

>>Autophagy - animal ;
>>Protein processing in endoplasmic reticulum ;
>>Apoptosis ;
>>Non-alcoholic fatty liver disease ;
>>Alzheimer disease ;
>>Parkinson disease ;
>>Amyotrophic lateral sclerosis ;
>>Huntington disease ;
>>Spinocerebellar ataxia ;
>>Pathways of neurodegeneration - multiple diseases ;
>>Lipid and atherosclerosis