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Gene Review

Ern1  -  endoplasmic reticulum (ER) to nucleus...

Mus musculus

Synonyms: 9030414B18Rik, AI225830, C85377, Endoplasmic reticulum-to-nucleus signaling 1, IRE1a, ...
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Disease relevance of Ern1


High impact information on Ern1

  • In yeast, the transmembrane protein kinase/endoribonuclease Ire1p activated by endoplasmic reticulum stress cleaves HAC1 mRNA, leading to production of the transcription factor Hac1p that activates the unfolded protein response (UPR) [5].
  • Three ER transmembrane protein kinases (Ire1alpha, Ire1beta, and PERK) have been implicated as proximal effectors of the mammalian UPR [6].
  • Activated Ire1 initiates an ER stress response pathway, and mouse ameloblasts were shown to express activated Ire1 [1].
  • The mammalian unfolded protein response (UPR) includes two major branches: one(s) specific to ER stress (Ire1/XBP-1 and ATF6-dependent), and one(s) shared by other cellular stresses (PERK/eIF-2alpha phosphorylation-dependent) [7].
  • PERK and Ire1alpha and eIF2alpha are also activated in calreticulin-deficient cells [8].

Biological context of Ern1


Anatomical context of Ern1

  • Characterization of mouse Ire1 alpha: cloning, mRNA localization in the brain and functional analysis in a neural cell line [2].

Analytical, diagnostic and therapeutic context of Ern1


  1. Fluoride induces endoplasmic reticulum stress in ameloblasts responsible for dental enamel formation. Kubota, K., Lee, D.H., Tsuchiya, M., Young, C.S., Everett, E.T., Martinez-Mier, E.A., Snead, M.L., Nguyen, L., Urano, F., Bartlett, J.D. J. Biol. Chem. (2005) [Pubmed]
  2. Characterization of mouse Ire1 alpha: cloning, mRNA localization in the brain and functional analysis in a neural cell line. Miyoshi, K., Katayama, T., Imaizumi, K., Taniguchi, M., Mori, Y., Hitomi, J., Yui, D., Manabe, T., Gomi, F., Yoneda, T., Tohyama, M. Brain Res. Mol. Brain Res. (2000) [Pubmed]
  3. Mouse hepatitis virus infection activates the Ire1/XBP1 pathway of the unfolded protein response. Bechill, J., Chen, Z., Brewer, J.W., Baker, S.C. Adv. Exp. Med. Biol. (2006) [Pubmed]
  4. Activation of hepatitis B virus S promoter by a cell type-restricted IRE1-dependent pathway induced by endoplasmic reticulum stress. Huang, Z.M., Tan, T., Yoshida, H., Mori, K., Ma, Y., Yen, T.S. Mol. Cell. Biol. (2005) [Pubmed]
  5. XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor. Yoshida, H., Matsui, T., Yamamoto, A., Okada, T., Mori, K. Cell (2001) [Pubmed]
  6. PERK mediates cell-cycle exit during the mammalian unfolded protein response. Brewer, J.W., Diehl, J.A. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  7. Herp is dually regulated by both the endoplasmic reticulum stress-specific branch of the unfolded protein response and a branch that is shared with other cellular stress pathways. Ma, Y., Hendershot, L.M. J. Biol. Chem. (2004) [Pubmed]
  8. Compromised calnexin function in calreticulin-deficient cells. Knee, R., Ahsan, I., Mesaeli, N., Kaufman, R.J., Michalak, M. Biochem. Biophys. Res. Commun. (2003) [Pubmed]
  9. Disturbed activation of endoplasmic reticulum stress transducers by familial Alzheimer's disease-linked presenilin-1 mutations. Katayama, T., Imaizumi, K., Honda, A., Yoneda, T., Kudo, T., Takeda, M., Mori, K., Rozmahel, R., Fraser, P., George-Hyslop, P.S., Tohyama, M. J. Biol. Chem. (2001) [Pubmed]
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