X-box binding protein 1

Authors:Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Guido Gerken

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Last updated on: 29.10.2020

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Synonym(s)

OMIM: 612371; X BOX-BINDING PROTEIN 2; XBP1; XBP2

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DefinitionThis section has been translated automatically.

XBP1 is the acronym for "X-Box-Binding Protein 1", a protein encoding the gene of the same name located on chromosome 22q12.1.

General informationThis section has been translated automatically.

The expression of the XBP1 protein is controlled by the cytokine IL-4, among others. XBP1 in turn controls the expression of IL-6, a cytokine involved in the growth and differentiation of plasma cells. As part of theunfolded protein response, the XBP1 mRNA is unconventionally spliced in the cytoplasm to produce a mature mRNA. The unspliced XBP1 mRNA encodes XBP1U, a protein that negatively regulates XBP1S and terminates the ER stress response (AragonT et al. (2009). A deletion of Xbp1 induces Paneth cell-specific autophagy and can lead to spontaneous ileitis (Adolph et al. 2013).

Note(s)This section has been translated automatically.

Unfolded Protein Response" is a protective mechanism of the cell coupled to the endoplasmic reticulum (ER), which is caused by the accumulation of misfolded proteins in the endoplasmic reticulum (Kozutsumi Y et al. 1988). A misregulation of this protein quality control by UPR can be the cause of numerous diseases.

LiteratureThis section has been translated automatically.

  1. Adolph TE et al (w2013) Paneth cells as a site of origin for intestinal inflammation. Nature 503: 272-276
  2. AragonT et al (2009) Messenger RNA targeting to endoplasmic reticulum stress signalling sites. Nature 457: 736-740
  3. Calfon M et al (2002) IRE1 couples endoplasmic reticulum load to secretory capacity by processing the XBP-1 mRNA. Nature 415: 92-96
  4. Kozutsumi Y et al (1988) The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins. Nature 332: 462-464

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Last updated on: 29.10.2020