| Ero1aGene Symbol: Ero1a Description: endoplasmic reticulum oxidoreductase 1 alpha Alias: Ero1l, ERO1-like protein alpha, ERO1-L, ERO1-L-alpha, endoplasmic oxidoreductin-1-like protein, endoplasmic reticulum oxidoreductase alpha, endoplasmic reticulum oxidoreductin-1-like protein, global ischemia-induced protein 11, oxidoreductin-1-L-alpha Species: rat Cabibbo A, Pagani M, Fabbri M, Rocchi M, Farmery M, Bulleid N, et al. ERO1-L, a human protein that favors disulfide bond formation in the endoplasmic reticulum. J Biol Chem. 2000;275:4827-33 pubmed ..ERO1-L is no longer functional when either one of the highly conserved Cys-394 or Cys-397 is mutated. These results strongly suggest that ERO1-L is involved in oxidative ER protein folding in mammalian cells. .. Mezghrani A, Fassio A, Benham A, Simmen T, Braakman I, Sitia R. Manipulation of oxidative protein folding and PDI redox state in mammalian cells. EMBO J. 2001;20:6288-96 pubmed ..Mutants in the Ero1-Lalpha CXXCXXC motif act as dominant negatives by limiting immunoglobulin oxidation. PDI-dependent oxidative folding in living cells can thus be manipulated by using hERO variants. .. Marciniak S, Yun C, Oyadomari S, Novoa I, Zhang Y, Jungreis R, et al. CHOP induces death by promoting protein synthesis and oxidation in the stressed endoplasmic reticulum. Genes Dev. 2004;18:3066-77 pubmed ..CHOP deletion thus protects cells from ER stress by decreasing ER client protein load and changing redox conditions within the organelle. .. Li G, Mongillo M, Chin K, Harding H, Ron D, Marks A, et al. Role of ERO1-alpha-mediated stimulation of inositol 1,4,5-triphosphate receptor activity in endoplasmic reticulum stress-induced apoptosis. J Cell Biol. 2009;186:783-92 pubmed publisher ..this response is blocked by siRNA-mediated silencing of ERO1-alpha or IP3R1 and by loss-of-function mutations in Ero1a or Chop. Reconstitution of ERO1-alpha in Chop(-/-) macrophages restores ER stress-induced IICR and apoptosis... Zito E, Chin K, Blais J, Harding H, Ron D. ERO1-beta, a pancreas-specific disulfide oxidase, promotes insulin biogenesis and glucose homeostasis. J Cell Biol. 2010;188:821-32 pubmed publisher ..Surprisingly, concomitant disruption of Ero1l, encoding the other ERO1 isoform, ERO1-alpha, does not exacerbate the ERO1-beta deficiency phenotype... Zito E, Hansen H, Yeo G, Fujii J, Ron D. Endoplasmic reticulum thiol oxidase deficiency leads to ascorbic acid depletion and noncanonical scurvy in mice. Mol Cell. 2012;48:39-51 pubmed publisher ..Compromised ER disulfide relay thus exposes protein thiols to competing oxidation to sulfenic acid, resulting in depletion of ascorbic acid, impaired procollagen proline 4-hydroxylation, and a noncanonical form of scurvy. .. Luyckx V, Compston C, Simmen T, Mueller T. Accelerated senescence in kidneys of low-birth-weight rats after catch-up growth. Am J Physiol Renal Physiol. 2009;297:F1697-705 pubmed publisher ..03). Low-birth-weight rats exhibit accelerated senescence in kidneys and hearts after rapid catch-up growth, a likely important link between early growth and subsequent hypertension, renal, and cardiovascular disease. .. Marino M, Stoilova T, Giorgi C, Bachi A, Cattaneo A, Auricchio A, et al. SEPN1, an endoplasmic reticulum-localized selenoprotein linked to skeletal muscle pathology, counteracts hyperoxidation by means of redox-regulating SERCA2 pump activity. Hum Mol Genet. 2015;24:1843-55 pubmed publisher ..These observations reveal the involvement of SEPN1 in ER redox and calcium homeostasis and that an ERO1 inhibitor, restoring redox-dependent calcium homeostasis, may ameliorate the myopathy of SEPN1 deficiency. .. Gess B, Hofbauer K, Wenger R, Lohaus C, Meyer H, Kurtz A. The cellular oxygen tension regulates expression of the endoplasmic oxidoreductase ERO1-Lalpha. Eur J Biochem. 2003;270:2228-35 pubmed
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