ER chaperones in mammalian development and human diseases

Min Ni, Amy S. Lee

Research output: Contribution to journalArticle

552 Citations (Scopus)

Abstract

The field of endoplasmic reticulum (ER) stress in mammalian cells has expanded rapidly during the past decade, contributing to understanding of the molecular pathways that allow cells to adapt to perturbations in ER homeostasis. One major mechanism is mediated by molecular ER chaperones which are critical not only for quality control of proteins processed in the ER, but also for regulation of ER signaling in response to ER stress. Here, we summarized the properties and functions of GRP78/BiP, GRP94/gp96, GRP170/ORP150, GRP58/ERp57, PDI, ERp72, calnexin, calreticulin, EDEM, Herp and co-chaperones SIL1 and P58IPK and their role in development and diseases. Many of the new insights are derived from recently constructed mouse models where the genes encoding the chaperones are genetically altered, providing invaluable tools for examining the physiological involvement of the ER chaperones in vivo.

Original languageEnglish (US)
Pages (from-to)3641-3651
Number of pages11
JournalFEBS Letters
Volume581
Issue number19
DOIs
StatePublished - Jul 31 2007

Fingerprint

Human Development
Endoplasmic Reticulum
Calnexin
Calreticulin
Gene encoding
Endoplasmic Reticulum Stress
Quality control
Cells
Proteins
Quality Control
Homeostasis
Genes
endoplasmic reticulum glycoprotein p72
glucose-regulated proteins
glucose-regulated protein 170

Keywords

  • Chaperones
  • Diseases
  • Endoplasmic reticulum
  • Mammalian development

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

ER chaperones in mammalian development and human diseases. / Ni, Min; Lee, Amy S.

In: FEBS Letters, Vol. 581, No. 19, 31.07.2007, p. 3641-3651.

Research output: Contribution to journalArticle

Ni, Min ; Lee, Amy S. / ER chaperones in mammalian development and human diseases. In: FEBS Letters. 2007 ; Vol. 581, No. 19. pp. 3641-3651.
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